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Susanibar Mesías EE, León Jorba A, Raventós Estellé A, Schinder CA, Rodriguez Rubio D. Removal of a giant musculocutaneous nerve schwannoma under intraoperative neurophysiological monitoring: Case report video and review of the literature. NEUROCIRUGIA (ENGLISH EDITION) 2025:500667. [PMID: 40139270 DOI: 10.1016/j.neucie.2025.500667] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/04/2024] [Revised: 02/19/2025] [Accepted: 02/24/2025] [Indexed: 03/29/2025]
Abstract
Schwannomas of the musculocutaneous nerve (MCN) are rare benign tumors of the peripheral nerve sheath. Due to their slow growth, they are often diagnosed late. In the upper limbs, schwannomas typically affect longer peripheral nerves at a distal level, making MCN cases uncommon. Ultrasound (US) and magnetic resonance imaging (MRI) are essential tools for early detection. While most schwannomas can be surgically removed without damaging the nerve, intraoperative neurophysiological monitoring (IONM) is critical if fascicular involvement exists. We present a 73-year-old right-handed Jehovah's Witness with a history of mild polio affecting the right arm. MRI showed a slow-growing, cystic mass in the right biceps, originating from the MCN and suggestive of schwannoma. Surgery achieved gross total resection without nerve damage. Histopathology confirmed a cystic schwannoma. In atypical proximal upper limb tumors, MCN schwannoma should be considered, with US/MRI crucial for diagnosis. IONM-assisted removal can minimize postoperative complications.
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Affiliation(s)
| | - Alba León Jorba
- Servicio de Neurología, Sección de Neurofisiología, Hospital del Mar, Barcelona, Spain
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Yao WT, Hsu HH, Liang ML, Huang HY, Lao AHC, Yu CM, Tung KY, Chen HL, Yang SC, Tu CP, Tsai MF. Feasibility of Intraoperative Somatosensory Evoked Potential Monitoring for Resection of Brachial Plexus Schwannomas: A Pilot Study. Ann Plast Surg 2025; 94:S52-S57. [PMID: 39996543 DOI: 10.1097/sap.0000000000004204] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/26/2025]
Abstract
OBJECTIVES The complex anatomical location of brachial plexus tumors is a formidable diagnostic and therapeutic challenge for surgeons. Intraoperative neurophysiologic monitoring enables the surgeon to proceed with more aggressive maneuvers for near total removal of the spinal cord tumor, such as somatosensory evoked potentials (SSEPs) monitoring. The present study aimed to describe the SSEP-assisted microsurgical excision technique and to evaluate the surgical outcomes of brachial plexus schwannomas. METHODS Thirteen patients who underwent SSEP-guided microsurgery for brachial plexus schwannomas between October 2011 and December 2020 were included in our study. RESULTS The mean age of the patients was 50 years (range, 29-64 years), with six tumors localized on the right, six on the left side and one on bilateral sides. Eight patients had preoperative motor/sensory dysfunction. Compared to preoperative Visual Analogue Scale (VAS) pain score, the postoperative VAS score significantly decreased in these patients (P = 0.005). No postoperative neurovascular injury was observed in the patients. Among patients who had a preoperative neurologic deficit, these preoperative neurologic deficits were relieved or improved after surgery. There was a low disability in the arm, shoulder, and hand after surgery. CONCLUSIONS Brachial plexus schwannomas is able to completely excised by SSEP-assisted delicately surgical techniques, contributing to a surgical benefit for the excision of brachial plexus schwannomas, particularly in patients with lower trunk involvement.
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Affiliation(s)
| | - Hsiu-Han Hsu
- From the Division of Plastic Surgery, Department of Surgery, Mackay Memorial Hospital
| | | | - Hsuan-Yu Huang
- From the Division of Plastic Surgery, Department of Surgery, Mackay Memorial Hospital
| | - Amy Hsuan-Chih Lao
- Pain Management Center, Department of Anesthesiology, Mackay Memorial Hospital, Taipei, Taiwan
| | - Chia-Meng Yu
- From the Division of Plastic Surgery, Department of Surgery, Mackay Memorial Hospital
| | - Kwang-Yi Tung
- From the Division of Plastic Surgery, Department of Surgery, Mackay Memorial Hospital
| | - Hsuen-Li Chen
- Department of Materials Science and Engineering, National Taiwan University
| | - Shan-Chiao Yang
- Department of Materials Science and Engineering, National Taiwan University
| | - Chih-Peng Tu
- From the Division of Plastic Surgery, Department of Surgery, Mackay Memorial Hospital
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de Matos PR, Silva M, Rosa G, Canão P, Azevedo F. Atypical presentation of Schwannoma mimicking squamous cell carcinoma. An Bras Dermatol 2025; 100:360-362. [PMID: 39848834 PMCID: PMC11962892 DOI: 10.1016/j.abd.2024.07.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2024] [Revised: 07/10/2024] [Accepted: 07/22/2024] [Indexed: 01/25/2025] Open
Affiliation(s)
| | - Miguel Silva
- Department of Dermatology and Venereology, ULS São João, Porto, Portugal
| | - Gilberto Rosa
- Department of Dermatology and Venereology, ULS São João, Porto, Portugal
| | - Pedro Canão
- Department of Pathology, ULS São João, Porto, Portugal
| | - Filomena Azevedo
- Department of Dermatology and Venereology, ULS São João, Porto, Portugal
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Spatz M, O'Donnell M, Gamil A, Gerstman B. Intramuscular schwannoma presenting as treatment-resistant lumbar radiculopathy: Case report. INTERVENTIONAL PAIN MEDICINE 2025; 4:100560. [PMID: 40124673 PMCID: PMC11929105 DOI: 10.1016/j.inpm.2025.100560] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 01/12/2025] [Revised: 02/13/2025] [Accepted: 02/16/2025] [Indexed: 03/25/2025]
Abstract
Intramuscular schwannomas are rare tumors originating from Schwann cells in the peripheral nervous system. These tumors typically occur along small motor nerves deep within the muscle, presenting diagnostic challenges for clinicians due to their rarity and nonspecific symptoms. They can induce pain that is resistant to interventions, and surgical removal is often the only method that provides relief of symptoms. We present a case of a 65-year-old male with low back pain and concurrent right anterior thigh pain and paresthesia. Despite extensive evaluation by numerous specialists and thorough diagnostic testing, including spinal MRIs and electrodiagnostic studies, no clear diagnosis was initially established. His symptoms remained resistant to medical and interventional treatments. Ultimately, MRI imaging of the right thigh revealed a mass measuring approximately 2 cm in the vastus medialis. Following excision of the mass, the patient experienced immediate symptom relief. This case highlights the importance of maintaining a broad differential when evaluating low back pain with associated lower extremity symptoms. We aim to highlight the importance of recognizing this possibility of extra-spinal pain generators.
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Affiliation(s)
- Moshe Spatz
- Department of Physical Medicine and Rehabilitation, Rutgers, New Jersey Medical School, Newark, NJ, USA
| | - Madison O'Donnell
- New York Institute of Technology College of Osteopathic Medicine, Glen Head, NY, USA
| | - Amir Gamil
- Rutgers, New Jersey Medical School, Newark, NJ, USA
| | - Brett Gerstman
- Department of Physical Medicine and Rehabilitation, Rutgers, New Jersey Medical School, Newark, NJ, USA
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Girgis F, Adam E, Suppiah S, Umansky D, Midha R. C2 Ganglionectomy for Pain Associated with Multiple Occipital Neurofibromas. Can J Neurol Sci 2024:1-2. [PMID: 39268615 DOI: 10.1017/cjn.2024.282] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/17/2024]
Affiliation(s)
- Fady Girgis
- Department of Neurosurgery, University of Calgary, Calgary, AB, Canada
| | - Elizabeth Adam
- Department of Neurosurgery, University of Calgary, Calgary, AB, Canada
| | - Suganth Suppiah
- Department of Neurosurgery, University of Toronto, Toronto, ON, Canada
| | - Daniel Umansky
- Department of Neurosurgery, University of Calgary, Calgary, AB, Canada
- Department of Neurosurgery, University of Utah Health, Salt Lake City, UT, USA
| | - Rajiv Midha
- Department of Neurosurgery, University of Calgary, Calgary, AB, Canada
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Puhaindran ME, Lim AYT. Tumors of the Brachial Plexus: A Critical Analysis Review. JBJS Rev 2024; 12:01874474-202406000-00001. [PMID: 38875357 PMCID: PMC11175764 DOI: 10.2106/jbjs.rvw.24.00019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/16/2024]
Abstract
» Tumors of the brachial plexus are uncommon and can present as a mass, with or without neurological symptoms. At times, asymptomatic tumors are also picked up incidentally when imaging is performed for other reasons.» Magnetic resonance imaging is the main imaging modality used to evaluate tumors of the brachial plexus. Other imaging modalities can be used as required.» Benign tumors that are asymptomatic should be observed. Excision can be considered for those that are found to be growing over time.» Biopsies of tumors of the brachial plexus are associated with the risk of nerve injury. Despite this, they should be performed for tumors that are suspected to be malignant before starting definitive treatment.» For malignant tumors, treatment decisions should be discussed at multidisciplinary tumor boards, and include both the oncology and peripheral nerve surgical team, musculoskeletal radiology, neuroradiology, and general radiology.
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Affiliation(s)
- Mark Edward Puhaindran
- Department of Hand and Reconstructive Microsurgery, National University Hospital, Singapore
| | - Aymeric Yu Tang Lim
- Department of Hand and Reconstructive Microsurgery, National University Hospital, Singapore
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Cooper-Khan RS, Frankovich AN, Thompson CA, Thomovsky SA, Lewis MJ. Clinical Findings and Outcome in 30 Dogs with Presumptive or Confirmed Nerve Sheath Tumors. Vet Sci 2024; 11:192. [PMID: 38787164 PMCID: PMC11125868 DOI: 10.3390/vetsci11050192] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2024] [Revised: 03/28/2024] [Accepted: 04/26/2024] [Indexed: 05/25/2024] Open
Abstract
Nerve sheath tumors (NSTs) are well-recognized primary nervous system tumors, but there is relatively limited information in dogs including comparison of NSTs in different anatomical locations. This retrospective study describes the clinical features and outcomes in a group of dogs with NSTs affecting the cranial nerves or spinal nerves. Thirty dogs were included, 25 with a presumptive diagnosis and five confirmed by histopathologic analysis. Seven dogs also had cytology of tumor samples, which were supportive of the NST diagnosis in four. Eight dogs had cranial nerve-associated NSTs, with six involving the trigeminal nerve. Twenty-two dogs had spinal nerve-associated NSTs including 13 invading the spinal canal and nine peripheral to the spinal canal, with the majority affecting nerves or nerve roots of the brachial plexus. The prognosis was poor, with dogs being euthanized eventually because of disease progression. Among dogs alive 1 week after diagnosis, the median survival time was 4 months but ranged from 2 weeks to >2 years. While there was a broad overlap between NST locations, survival was generally longer for dogs without spinal canal or intracranial involvement. The results expand available information on NSTs in dogs but should be interpreted with caution given the small number of dogs with a definitive diagnosis. Further investigation is warranted to determine how tumor location, invasiveness, and treatments pursued impact outcome.
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Affiliation(s)
- Rachel S. Cooper-Khan
- Department of Veterinary Clinical Sciences, Purdue University College of Veterinary Medicine, 625 Harrison Street, West Lafayette, IN 47907, USA; (R.S.C.-K.); (S.A.T.)
| | - Alexandra N. Frankovich
- Department of Comparative Pathobiology, Purdue University College of Veterinary Medicine, 625 Harrison Street, West Lafayette, IN 47907, USA; (A.N.F.); (C.A.T.)
| | - Craig A. Thompson
- Department of Comparative Pathobiology, Purdue University College of Veterinary Medicine, 625 Harrison Street, West Lafayette, IN 47907, USA; (A.N.F.); (C.A.T.)
| | - Stephanie A. Thomovsky
- Department of Veterinary Clinical Sciences, Purdue University College of Veterinary Medicine, 625 Harrison Street, West Lafayette, IN 47907, USA; (R.S.C.-K.); (S.A.T.)
| | - Melissa J. Lewis
- Department of Veterinary Clinical Sciences, Purdue University College of Veterinary Medicine, 625 Harrison Street, West Lafayette, IN 47907, USA; (R.S.C.-K.); (S.A.T.)
- Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, 1060 William Moore Drive, Raleigh, NC 27606, USA
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Öhlén E, El-Hajj VG, Fletcher-Sandersjöö A, Edström E, Elmi-Terander A. Clinical course and predictors of outcome following surgical treatment of benign peripheral nerve sheath tumors, a single center retrospective study. Int J Neurosci 2024:1-7. [PMID: 38618859 DOI: 10.1080/00207454.2024.2342977] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2023] [Accepted: 04/09/2024] [Indexed: 04/16/2024]
Abstract
INTRODUCTION Peripheral nerve sheath tumors are the most common tumor of the peripheral nerves. In general, surgery has a favorable outcome and is the treatment of choice. However, postoperative neurologic deficits are not uncommon, and predictors of outcome are poorly defined. OBJECTIVE To evaluate clinical outcomes after surgical treatment of benign peripheral nerve sheath tumors and identify outcome predictors that may affect preoperative decision making and improve surgical outcomes. METHOD In this single center retrospective study, all patients surgically treated for a benign peripheral nerve sheath tumor between 2005 and 2020 were eligible for inclusion. Medical records and imaging data were reviewed. Studied outcomes were changes in neurological symptoms, pain, and tumor recurrence. Logistic regression was performed to identify possible outcome predictors. RESULTS In total, 81 patients undergoing 85 separate surgeries for benign peripheral nerve sheath tumors were included. The most common preoperative symptoms were local pain (90%) followed by a noticeable mass (78%), radiating pain (72%), sensory deficit (18%), and motor deficit (16%). A postoperative improvement of symptoms was seen in 94% of those with pain, 48% of those with sensory deficits and 78% of those with motor deficits. However, 35% and 9% developed new postoperative sensory and motor deficits, respectively. Multivariable analysis showed complete tumor removal as a predictor of reduced pain (p = 0.033), and younger age and larger tumors were risk factors for persistent or increased sensory deficits (p = 0.002 and p = 0.005, respectively). There were no significant predictors of motor deficits. Neurocutaneous syndromes were associated with increased odds of tumor recurrence on univariable analysis (p = 0.008). CONCLUSION Surgery of benign peripheral nerve sheath tumors is a safe procedure with a favorable outcome in most cases. Younger age and larger tumors were risk factors for persistent or increased sensory deficits, while complete tumor removal was associated with reduced pain. Patients with neurocutaneous syndromes had a higher rate of tumor recurrence. To further evaluate outcome predictors, we recommend future studies to focus on longer follow-up periods to assess the natural course of postoperative neurological deficits.
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Affiliation(s)
- Erik Öhlén
- Department of Pediatrics, Mälar Hospital, Eskilstuna, Sweden
- Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden
| | | | | | - Erik Edström
- Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden
- Capio Spine Center Stockholm, Löwenströmska Hospital, Upplands-Väsby, Sweden
- Department of Medical Sciences, Örebro University, Örebro, Sweden
| | - Adrian Elmi-Terander
- Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden
- Capio Spine Center Stockholm, Löwenströmska Hospital, Upplands-Väsby, Sweden
- Department of Medical Sciences, Örebro University, Örebro, Sweden
- Department of Surgical Sciences, Uppsala University, Uppsala, Sweden
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Verly G, Delfino T, Oliveira LDB, Batista S, Sousa MP, Schevz R, Ferreira MY, Bertani R. A light in the darkness: sodium fluorescein-assisted peripheral nerve sheath tumors resection - a comprehensive systematic review and single-arm meta-analysis. Neurosurg Rev 2024; 47:181. [PMID: 38649501 DOI: 10.1007/s10143-024-02414-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2023] [Revised: 02/13/2024] [Accepted: 04/09/2024] [Indexed: 04/25/2024]
Abstract
The treatment for peripheral nerve sheath tumors (PNSTs) is based on surgical excision and the primary goal is to improve symptoms whilst preserving neurological function. In order to improve this technique, surgeons may use sodium fluorescein (SF) to help visualize the neoplasm and, consequently, facilitate its removal. Aiming to assess the efficacy of this emerging surgical strategy, we conducted a systematic review and single-arm meta-analysis. We conducted a systematic search on the PubMed, Embase, and Web of Science databases, following the PRISMA guidelines. Studies without outcomes of interest, case series with less than four patients, letters, comments, technical notes, editorials, reviews, and basic research papers were excluded. The outcomes considered for this study were: the number of tumors that achieved total resection, subtotal resection, or near total resection, the approach/technique utilized by the surgeon, SF-related complications, and total complications. Five studies, with a total of 175 individuals, were included in our survey. Notably, 70% of the neoplasms presented by the patients were schwannomas. Considering extracranial lesions, we found a proportion of 96% (95% CI: 88 - 100%) in total resection, 0% (95% CI: 0-1%) in near total resection, and 4% (95% CI: 0-12%) in subtotal resection, all linked to an amount of 185 analyzed PNSTs. Furthermore, a proportion of 1% (95% CI: 0 - 2%) in SF-related complications was spotted among 183 patients. Finally, total complications analysis accounted for 11% (95% CI: 0 - 25%) among 183 individuals. We concluded that SF-assisted resection of PNSTs is a suitable and relatively safe technique, linked to minimum complications, of which the majority was not associated with the chemical compound itself. Future research is necessary to increase the number of patients available in the current literature and, therefore, enhance future analyses.
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Affiliation(s)
- Gabriel Verly
- Faculty of Medicine, Federal University of Rio de Janeiro, R. Prof. Rodolpho Paulo Rocco, 255 - Cidade Universitária da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, 21941-617, Brazil
| | - Thiffany Delfino
- Faculty of Medicine, Federal University of Rio de Janeiro, R. Prof. Rodolpho Paulo Rocco, 255 - Cidade Universitária da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, 21941-617, Brazil
| | | | - Sávio Batista
- Faculty of Medicine, Federal University of Rio de Janeiro, R. Prof. Rodolpho Paulo Rocco, 255 - Cidade Universitária da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, 21941-617, Brazil
| | - Marcelo Porto Sousa
- Faculty of Medicine, Federal University of Rio de Janeiro, R. Prof. Rodolpho Paulo Rocco, 255 - Cidade Universitária da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, 21941-617, Brazil.
| | - Rodrigo Schevz
- Faculty of Medicine, Faculty of Medicine of ABC, Santo André, SP, Brazil
| | | | - Raphael Bertani
- Department of Neurosurgery, University of São Paulo, São Paulo, SP, Brazil
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Grübel N, Antoniadis G, AK U, Mayer B, König R, Wirtz CR, Pala A, Dengler NF, Pedro MT. Health-related quality of life in patients with peripheral nerve tumors: results from the German multicentric Peripheral Nerve Tumor Registry. Front Oncol 2024; 14:1398252. [PMID: 38711847 PMCID: PMC11070577 DOI: 10.3389/fonc.2024.1398252] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2024] [Accepted: 03/25/2024] [Indexed: 05/08/2024] Open
Abstract
Objective Peripheral nerve tumors (PNTs) are rare diseases. So far, no multicenter data on diagnostics, the efficacy of treatment, long-term outcomes, and health-related quality of life (HRQoL) exist. The establishment of the Peripheral Nerve Tumor Registry (PNTR) in 2015 allows for the systematic analysis of patients with tumors associated with peripheral nerves. The present study aims to investigate the impact of PNT on an individual's HRQoL and the effect of surgery. Methods HRQoL was pre- and postoperatively assessed by the Euro-Qol-5D-5L (EQ-5D-5L) and Euro-Qol visual analog scale (EQ-VAS) survey in the retrospective and prospective study arm in three active participating study centers. An index was calculated based on the EQ-5D-5L for the quantification of health state (0: worst possible state of health, 1: best possible state of health). The EQ-VAS ranges from 0% (worst imaginable health status) to 100% (best possible health status). Patient characteristics (age, sex), as well as disease (histopathological entity) and treatment (pre- and postoperative symptoms, type of treatment)-specific data, were analyzed. Results Data from 171 patients from three high-volume centers were included, with schwannoma (70.8%, n = 121) and neurofibroma (15.8%, n = 27) being the most prevalent histopathological diagnoses. Both the median health index value (preoperative: 0.887, n = 167; postoperative: 0.910, n = 166) and the median EQ-VAS (preoperative: 75%, n = 167; postoperative: 85%, n = 166) of the entire cohort regarding all histopathological diagnosis improved significantly after surgical therapy (p < 0.001). Preoperatively, 12.3% (n = 21) reached the highest index score of 1.0 in EQ-5D-5L and 100% in the EQ-VAS score in 5.3% (n = 9) of all patients. Postoperatively, the highest index score of 1.0 and 100% in the EQ-VAS score increased significantly and were achieved in 33.3% (n = 57) and 11.1% (n = 19) of the patients, respectively (p < 0.001). Conclusion For the first time, our study presents multicenter data on life quality and the effect of surgery in primarily benign peripheral nerve tumors. Early surgery at a specialized center could improve neurological outcomes and, in conclusion, better QoL. In summary, surgical therapy significantly improved the entire cohort's QoL, VAS, and analgesia.
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Affiliation(s)
- Nadja Grübel
- Peripheral Nerve Unit, Department of Neurosurgery, University Medicine of Ulm, Günzburg, Germany
| | - Gregor Antoniadis
- Peripheral Nerve Unit, Department of Neurosurgery, University Medicine of Ulm, Günzburg, Germany
| | - Uerschels AK
- Department of Neurosurgery, University Medicine Essen, Essen, Germany
| | - Benjamin Mayer
- Institute of Epidemiology and Medical Biometry, University of Ulm, Ulm, Germany
| | - Ralph König
- Peripheral Nerve Unit, Department of Neurosurgery, University Medicine of Ulm, Günzburg, Germany
| | - Christian Rainer Wirtz
- Peripheral Nerve Unit, Department of Neurosurgery, University Medicine of Ulm, Günzburg, Germany
| | - Andrej Pala
- Peripheral Nerve Unit, Department of Neurosurgery, University Medicine of Ulm, Günzburg, Germany
| | - Nora F. Dengler
- Department of Neurosurgery, University Medicine Charité Berlin, Berlin, Germany
| | - Maria Teresa Pedro
- Peripheral Nerve Unit, Department of Neurosurgery, University Medicine of Ulm, Günzburg, Germany
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Debs P, Luna R, Fayad LM, Ahlawat S. MRI features of benign peripheral nerve sheath tumors: how do sporadic and syndromic tumors differ? Skeletal Radiol 2024; 53:709-723. [PMID: 37845504 DOI: 10.1007/s00256-023-04479-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2023] [Revised: 09/20/2023] [Accepted: 10/06/2023] [Indexed: 10/18/2023]
Abstract
OBJECTIVES To compare MRI features of sporadic and neurofibromatosis syndrome-related localized schwannomas and neurofibromas. METHODS In this retrospective study, our pathology database was searched for "neurofibroma" or "schwannoma" from 2014 to 2019. Exclusion criteria were lack of available MRI and intradural or plexiform tumors. Qualitative and quantitative anatomic (location, size, relationship to nerve, signal, muscle denervation) and functional (arterial enhancement, apparent diffusion-weighted coefficient) MRI features of sporadic and syndrome-related tumors were compared. Statistical significance was assumed for p < 0.05. RESULTS A total of 80 patients with 64 schwannomas (sporadic: 42 (65.6%) v. syndrome-related: 22 (34.4%)) and 19 neurofibromas (sporadic: 7 (36.8%) v. syndrome-related: 12 (41.7%)) were included. Only signal heterogeneity (T2W p=0.001, post-contrast p=0.03) and a diffused-weighted imaging target sign (p=0.04) were more frequent with schwannomas than neurofibromas. Sporadic schwannomas were similar in size to syndrome-related schwannomas (2.9±1.2cm vs. 3.7±3.2 cm, p = 0.6), but with greater heterogeneity (T2W p = 0.02, post-contrast p = 0.01). Sporadic neurofibromas were larger (4.6±1.5cm vs. 3.4±2.4 cm, p = 0.03) than syndrome-related neurofibromas, also with greater heterogeneity (T2W p=0.03, post-contrast p=0.04). Additional tumors along an affected nerve were only observed with syndrome-related tumors). There was no difference in apparent diffusion coefficient values or presence of early perfusion between sporadic and syndrome-related tumors (p > 0.05). CONCLUSIONS Although syndrome-related and sporadic schwannomas and neurofibromas overlap in their anatomic, diffusion and perfusion features, signal heterogeneity and presence of multiple lesions along a nerve are differentiating characteristics of syndrome-related tumors.
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Affiliation(s)
- Patrick Debs
- The Russell H. Morgan Department of Radiology & Radiological Science, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD, 21287, USA.
| | - Rodrigo Luna
- The Russell H. Morgan Department of Radiology & Radiological Science, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD, 21287, USA
| | - Laura M Fayad
- The Russell H. Morgan Department of Radiology & Radiological Science, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD, 21287, USA
- Division of Orthopaedic Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
| | - Shivani Ahlawat
- The Russell H. Morgan Department of Radiology & Radiological Science, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD, 21287, USA
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Sulli D, Shankar C, Raikar SG. Peripheral Nerve Sheath Tumor: A Diagnostic and Therapeutic Challenge. Cureus 2024; 16:e56601. [PMID: 38646284 PMCID: PMC11031624 DOI: 10.7759/cureus.56601] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/11/2024] [Indexed: 04/23/2024] Open
Abstract
INTRODUCTION Peripheral nerve tumors are a group of rare soft tissue tumors of neuro-ectodermal origin. Although the majority of them are benign in nature, up to 10% can be malignant. The symptoms depend on the site, size, and structures compressed by the tumor. AIM To highlight the heterogeneity of signs and symptoms and their presentations, which has often made it difficult for the attending physician to accurately diagnose and direct the patient toward appropriate treatment. METHODS Eight patients treated at our tertiary care hospital between 2015 and 2022 were included in this study. They were evaluated in detail. Treatment was surgical. The patients underwent complete excision of the tumor under magnification to help preserve the adjacent neurovascular bundle. All patients were followed up post-operatively to document the status of their symptoms. RESULTS The average duration prior to referral to our hospital was 13 months. Seven subjects had pain at presentation, one had neurological deficit. Seven also complained of swelling. Five of the eight lesions were schwannoma, two neurofibroma and one showed malignant histology. Post-operatively, Hoffman Tinel signs improved in all six subjects. five of the seven subjects were completely pain-free, and the other two had a reduction in symptoms. CONCLUSIONS Early diagnosis and referral to a specialist center are needed to achieve satisfactory outcomes while treating peripheral nerve tumors. Proliferative lesions should be treated surgically in specialist centers by experienced doctors with appropriate skills and equipment for microsurgical procedures to ensure full recovery.
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Affiliation(s)
| | - Chandni Shankar
- Plastic and Reconstructive Surgery, Yenepoya Medical College, Mangalore, IND
| | - Shruti G Raikar
- Plastic and Reconstructive Surgery, Yenepoya Medical College, Mangalore, IND
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Friedrich RE, Löhmann DM. Neurofibromatosis type 1-associated plexiform neurofibromas of the neck: topography of lesions and surgical treatment data of 69 patients. Oral Maxillofac Surg 2024; 28:393-404. [PMID: 37173460 PMCID: PMC10914861 DOI: 10.1007/s10006-023-01155-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2022] [Accepted: 04/19/2023] [Indexed: 05/15/2023]
Abstract
PURPOSE Plexiform neurofibromas (PNF) are rare tumors arising from peripheral nerve sheath cells. PNF are a hallmark in patients with neurofibromatosis type 1 (NF1), a tumor predisposition syndrome. PNF often grow invasively and destructively, what may complicate surgical treatment. Data on frequency, location, and surgical procedures of patients with NF1-associated FPNF are scarce. This study provides treatment data of NF1 patients. METHODS Localization and treatment data of 69 NF1 patients with neck PNF were analyzed. Frequency of lesions was recorded in coded colors on schematic neck drawings. RESULTS The tumors showed no side preference, were located in the entire area under investigation, and did not respect anatomical units/dermatomes. However, the sternocleidomastoid region was particularly frequently affected. The mean number of surgical measures per patient was 1.33. Complications were extensive swelling, hematoma, and bleeding. Histological assessment usually confirmed the clinical assessment of neoplasm. However, histologic differentiation of PNST reveals differences in between tumors that have been unified in clinical assessment as PNF. CONCLUSION The color-coded, schematic overview of the frequency distribution of surgical neck interventions in NF1 patients with PNF proved a useful tool to gain assessment of preferred treatment needs. The imaging procedure may be suitable for controlling the external aspect of natural tumor development (growth, effects of aging) in the same way as the documentation of the post-surgical course. Treatment plans for patients with these tumors should consider that repeated interventions may be necessary to achieve a longer-term stable result.
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Affiliation(s)
- Reinhard E Friedrich
- Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Martinistraße 52, 20246, Hamburg, Germany.
| | - Daniel M Löhmann
- Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Martinistraße 52, 20246, Hamburg, Germany
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14
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Dengler NF, Pedro MT, Kolbenschlag J. [Interdisciplinary Treatment Of Tumorous And Tumour-Like Lesions Of Peripheral Nerves]. HANDCHIR MIKROCHIR P 2024; 56:11-20. [PMID: 38508203 DOI: 10.1055/a-2250-7772] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/22/2024] Open
Abstract
Tumorous or tumour-like lesions of peripheral nerves are generally rare, heterogeneous and challenging to diagnose and treat. They may become apparent by a palpable swelling (lump) near nerves, sensory and/or motor deficits, pain to touch or neuropathic pain. In 91% of cases, tumours are benign. The differentiation of entities and their characteristics as well as a function-preserving resection strategy are highly relevant. Misdiagnosis and inadequate treatment can lead to severe deficits and pain syndromes. Benign tumours include schwannomas and neurofibromas, which can occur sporadically but can also be associated with neurogenetic tumour disposition syndromes if they occur more frequently. Rarer benign nerve tumours include perineuriomas, lipomas, aggressive fibrosis (desmoid tumours), paragangliomas and haemangiomas. Ganglion cysts are described as tumour-like lesions. The association of nerve tumours with neurogenetic syndromes and the correct classification of potentially malignant lesions such as MPNST (malignant peripheral nerve sheath tumour) or intermediate stages such as ANNUBPs (atypical neurofibromatous neoplasms with unknown biological potential) pose particular challenges. Interdisciplinarity is highly relevant for clinical treatment and a correct diagnosis. The aim of our work is to provide an overview of the relevant entities, diagnostic evaluation and contemporary treatment strategies based on the current data situation and taking into account the recently published interdisciplinary AWMF S2k guideline "Diagnosis and Treatment of Peripheral Nerve Tumours".
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Affiliation(s)
- Nora Franziska Dengler
- Fakultät für Gesundheitswissenschaften Brandenburg, Medizinische Hochschule Theodor Fontane, Bad Saarow, Germany
- Klinik für Neurochirurgie, Helios Klinik Bad Saarow, Germany
| | - Maria Teresa Pedro
- Sektion für Periphere Nervenchirurgie, Klinik für Neurochirurgie, Univeristätsklinikum Ulm am BKH Günzburg, Ulm, Germany
| | - Jonas Kolbenschlag
- Universitätsklinik für Hand-, Plastische, Rekonstruktive und Verbrennungschirurgie, BG Klinik Tübingen, Tubingen, Germany
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15
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Ozden SB, Simsekoglu MF, Sertbudak I, Demirdag C, Gurses I. Epithelioid malignant peripheral nerve sheath tumor of the bladder and concomitant urothelial carcinoma: A case report. World J Clin Cases 2024; 12:551-559. [PMID: 38322457 PMCID: PMC10841956 DOI: 10.12998/wjcc.v12.i3.551] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/28/2023] [Revised: 08/08/2023] [Accepted: 08/17/2023] [Indexed: 01/18/2024] Open
Abstract
BACKGROUND Epithelioid malignant peripheral nerve sheath tumor (EMPNST) of the bladder is a rare entity with devastating features. These tumors are thought to originate from malignant transformation of pre-existing schwannomas of pelvic autonomic nerve plexuses, and unlike the conventional malignant peripheral nerve sheath tumor (MPNST), are not associated with neurofibromatosis. The tumor has distinctive morphological, immunohistochemical and molecular features. Additionally, it tends to be more aggressive and have a higher mortality. This is the first case that presents with a synchronous urothelial carcinoma of the bladder and the epithelioid variant of MPNST in the literature. It's also the second reported case of EMPNST originating from the bladder wall. CASE SUMMARY In this case report, we present the detailed clinical course of a 71-year-old patient with EMPNST of the bladder alongside a literature review. CONCLUSION During the management of EMPNST cases, offering aggressive treatment modalities to the patient, such as radical cystectomy, is appropriate for the best chance to contain the disease, regardless of the tumor stage and the extent of local disease at initial diagnosis.
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Affiliation(s)
- Sami Berk Ozden
- Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Urology, Istanbul 34100, Fatih, Turkey
| | - Muhammed Fatih Simsekoglu
- Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Urology, Istanbul 34100, Fatih, Turkey
| | - Ipek Sertbudak
- Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Pathology, Istanbul 34100, Fatih, Turkey
| | - Cetin Demirdag
- Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Urology, Istanbul 34100, Fatih, Turkey
| | - Iclal Gurses
- Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Pathology, Istanbul 34100, Fatih, Turkey
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16
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Giannini C, Righi A. Peripheral nerve tumors. HANDBOOK OF CLINICAL NEUROLOGY 2024; 201:251-271. [PMID: 38697744 DOI: 10.1016/b978-0-323-90108-6.00016-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/05/2024]
Abstract
The chapter is focused on the neoplastic peripheral nerve lesions, which primarily involve "cranial and paraspinal nerves," as outlined in the CNS volume (WHO_Classification_of_Tumours_Editorial_Board, 2021). These include classic peripheral nerve sheath tumors such as schwannoma, neurofibroma, intraneural perineurioma, and malignant peripheral nerve sheath tumors, with their variants as well as new and more precisely defined entities, including hybrid nerve sheath tumors and malignant melanotic nerve sheath tumor (previously melanotic schwannoma).
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Affiliation(s)
- Caterina Giannini
- Division of Anatomic Pathology, Laboratory Medicine/Pathology and Neurosurgery, Mayo Clinic College of Medicine, Mayo Clinic, Rochester, MN, United States; Department of Biomedical and Neuromotor Sciences (DIBINEM), Alma Mater Studiorum, University of Bologna, Bologna, Italy.
| | - Alberto Righi
- Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
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17
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Beecher G, Dyck PJB, Zochodne DW. Axillary and musculocutaneous neuropathies. HANDBOOK OF CLINICAL NEUROLOGY 2024; 201:135-148. [PMID: 38697736 DOI: 10.1016/b978-0-323-90108-6.00004-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/05/2024]
Abstract
This chapter covers axillary and musculocutaneous neuropathies, with a focus on clinically relevant anatomy, electrodiagnostic approaches, etiologic considerations, and management principles. Disorders of the lateral antebrachial cutaneous nerve, a derivative of the musculocutaneous nerve, are also reviewed. We emphasize the importance of objective findings, including the physical examination and electrodiagnostic evaluation in confirming the isolated involvement of each nerve which, along with the clinical history, informs etiologic considerations. Axillary and musculocutaneous neuropathies are both rare in isolation and most frequently occur in the setting of trauma. Less commonly encountered etiologies include external compression or entrapment, neoplastic involvement, or immune-mediated disorders including neuralgic amyotrophy, postsurgical inflammatory neuropathy, multifocal motor neuropathy, vasculitic neuropathy, and multifocal chronic inflammatory demyelinating polyradiculoneuropathy.
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Affiliation(s)
- Grayson Beecher
- Division of Neuromuscular Medicine, Department of Neurology, Mayo Clinic, Rochester, MN, United States; Neuroscience and Mental Health Institute and Division of Neurology, Department of Medicine, University of Alberta, Edmonton, AB, Canada
| | - P James B Dyck
- Division of Neuromuscular Medicine, Department of Neurology, Mayo Clinic, Rochester, MN, United States; Peripheral Neuropathy Research Laboratory, Mayo Clinic, Rochester, MN, United States
| | - Douglas W Zochodne
- Neuroscience and Mental Health Institute and Division of Neurology, Department of Medicine, University of Alberta, Edmonton, AB, Canada.
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18
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Chen R, Ran Y, Wu Y, Xu H, Niu J, Zhang Y, Cheng J. The value of the cinematic volume rendering technique: magnetic resonance imaging in diagnosing tumors associated with the brachial plexus. Eur J Med Res 2023; 28:569. [PMID: 38053185 DOI: 10.1186/s40001-023-01416-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2023] [Accepted: 09/30/2023] [Indexed: 12/07/2023] Open
Abstract
PURPOSE To examine the diagnostic advantages and clinical application value of the cinematic volume rendering technique (cVRT) when evaluating the relationship between the brachial plexus, peripheral tumor lesions, and blood vessels. MATERIALS AND METHODS Seventy-nine patients with brachial plexus tumors between November 2012 and July 2022 were enrolled in our study. All patients underwent T1WI, T2WI, three-dimensional short recovery time reversal recovery fast spin-echo imaging (3D-STIR-SPACE), and the T1WI enhancement sequence. In addition, cVRT was used to render and obtain a three-dimensional model that clearly showed the location and tissue structure of the brachial plexus nerves and the tumor in all directions. RESULTS Seventy-one patients (mean age, 47.1 years; 33 males, 38 females) with tumors around the brachial plexus were included in the study. The brachial plexus nerve, surrounding tumor lesions, and vascular anatomy of all patients were well displayed with cVRT. The tumors of 37 patients manifested as unilateral or bilateral growths along the brachial plexus nerve and were fusiform, spherical, or multiple beaded; seven patients' tumors pushed against the brachial plexus nerve and were circular, lobular, or irregular; sixteen patients' tumors encircled the brachial plexus nerve and were spherical; and eleven patients' tumors infiltrated the brachial plexus nerve and had irregular morphology. The mass has a moderately uniform or uneven signal on T1WI and a high or mixed signal on T2WI. After enhancement, the signal was evenly or unevenly strengthened. CONCLUSIONS cVRT clearly showed the origin of tumors associated with the brachial plexus and their relationship with the nerves and peripheral blood vessels, providing reliable information for clinical diagnosis and treatment.
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Affiliation(s)
- Rui Chen
- Department of Magnetic Resonance Imaging, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yuncai Ran
- Department of Magnetic Resonance Imaging, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yanglei Wu
- MR Collaborations, Siemens Healthineers Ltd, Beijing, China
| | - Haowen Xu
- Department of Interventional Neuroradiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Junxia Niu
- Department of Magnetic Resonance Imaging, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yong Zhang
- Department of Magnetic Resonance Imaging, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Jingliang Cheng
- Department of Magnetic Resonance Imaging, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
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19
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Ono R, Tominaga T, Nonaka T, Takamura Y, Oishi K, Shiraishi T, Hashimoto S, Noda K, Sawai T, Nagayasu T. Malignant peripheral nerve sheath tumor in the pelvis: a case report. Surg Case Rep 2023; 9:157. [PMID: 37672135 PMCID: PMC10482811 DOI: 10.1186/s40792-023-01733-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2023] [Accepted: 08/21/2023] [Indexed: 09/07/2023] Open
Abstract
BACKGROUND Malignant peripheral nerve sheath tumors (MPNSTs) are malignancies that arise or differentiate from or infiltrate peripheral nerves and account for approximately 5% of soft-tissue malignancies. Approximately half of MPNSTs develop in patients with neurofibromatosis type 1 (NF1), a hereditary disease. MPNSTs occur mainly in the trunk, proximal extremities, and neck, but can on rare occasion arise in or near the gastrointestinal tract, and intestinal complications have been reported. We describe herein a case with resection of an MPNST arising in the pelvic region. CASE PRESENTATION A 51-year-old woman had undergone repeated resections for systemic neurofibrosis associated with NF1. This time, a pelvic tumor was noted on follow-up positron emission tomography computed tomography (CT). She presented with slowly progressive radiating pain in the lower extremities and was referred to our hospital for tumor resection. Contrast-enhanced CT showed a 75 × 58-mm mass in the right greater sciatic foramen directly below a 24 × 28-mm mass. Open pelvic tumor resection was performed for pelvic neurofibroma. The obturator nerve was identified lateral to the main tumor and the sciatic nerve was identified dorsally, then dissection was performed. The closed nerve was spared, while the sciatic nerve was partially dissected and the two tumors were removed. Both tumors were elastic and hard. Pathologic findings were MPNST for the large specimen and neurofibroma with atypia for the small specimen. The patient developed temporary postoperative ileus, but is generally doing well and is currently free of recurrence or radiating pain. The patient is at high risk of recurrence and close monitoring should be continued. CONCLUSIONS We encountered a rare case of MPNST. Due to the high risk of recurrence, surgery with adequate margins was performed, with a requirement for appropriate follow-up.
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Affiliation(s)
- Rika Ono
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Tetsuro Tominaga
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
| | - Takashi Nonaka
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Yuma Takamura
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Kaido Oishi
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Toshio Shiraishi
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Shintaro Hashimoto
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Keisuke Noda
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Terumitsu Sawai
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
| | - Takeshi Nagayasu
- Department of Surgical Oncology, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
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20
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Uerschels AK, Dengler NF, Chihi M, Lenkeit A, Dinger TF, Jabbarli R, Sure U, Hagenacker T, Wrede KH, Gembruch O. Benign peripheral nerve sheath tumors: an interdisciplinary diagnostic and therapeutic challenge. Neurosurg Rev 2023; 46:205. [PMID: 37594583 PMCID: PMC10439084 DOI: 10.1007/s10143-023-02107-z] [Citation(s) in RCA: 7] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2023] [Revised: 07/27/2023] [Accepted: 08/01/2023] [Indexed: 08/19/2023]
Abstract
A benign peripheral nerve sheath tumor (bPNST) is a rare lesion associated with peripheral nerval structures. Symptoms may be heterogeneous, complicating diagnosis finding. Additionally, management concepts of bPNST may vary. In some cases, initial misdiagnosis leads to mistreatment resulting in severe functional deficits and chronic pain syndromes. Therefore, we analyzed patients treated for bPNST in our specialized institution with a primary focus on prior misdiagnosis and possible mistreatment. Patients with bPNSTs (schwannomas, neurofibromas, hybrid nerve sheath tumors, and perineuriomas) treated at the Neurosurgical Department between January 1, 2015, and July 31, 2021, were included. Assessment of demographics, tumor entity, tumor location, symptoms, the interval between the onset of symptoms and surgery, involved medical specialties, and outpatients' treatment, with particular focus on initial misdiagnosis and inappropriate medical treatment, was performed. Eighty-five patients were included in the final analysis with schwannoma being the most prevalent histopathological diagnosis (schwannoma (75.3%, n=64), neurofibroma (12.9%, n=11), hybrid nerve sheath tumor (5.9%, n=5), and perineurioma (5.9%, n=5)). An incorrect primary diagnosis was detected in 44.7% (n=38), leading to suboptimal or insufficient treatment in these cases. Of those, 28.9% (n=11/38) were treated suboptimal, while 18.5% (n=7/38) underwent unnecessary invasive diagnostics. Inappropriate surgery based on prior misdiagnosis, which led to severe neurological deficits in all these cases, was reported in 26.3% (n=10/38). For the first time, our data shows the quantity and impact of incorrect initial diagnosis in bPNST causing a delay in causative treatment or resulting in unnecessary or potentially harmful treatment.
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Affiliation(s)
- Anne-Kathrin Uerschels
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Nora F Dengler
- Department of Neurosurgery, Charité - Universitätsmedizin Berlin, Berlin, Germany
| | - Mehdi Chihi
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Annika Lenkeit
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Thiemo F Dinger
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Ramazan Jabbarli
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Ulrich Sure
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Tim Hagenacker
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Department of Neurology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Karsten H Wrede
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Oliver Gembruch
- Department of Neurosurgery and Spine Surgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.
- Center for Translational Neuro- & Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, Essen, Germany.
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21
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Pham Quang V, Hoang Quoc H, Nguyen B, Ngo Quang C, Nguyen Chi H, Nguyen N. Giant schwannoma on the lower leg: A case report and review of the literature. Int J Surg Case Rep 2023; 109:108468. [PMID: 37437325 PMCID: PMC10362252 DOI: 10.1016/j.ijscr.2023.108468] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2023] [Revised: 06/13/2023] [Accepted: 06/27/2023] [Indexed: 07/14/2023] Open
Abstract
INTRODUCTION AND IMPORTANCE Peripheral nerve sheath tumors are common neoplasm with different biological features ranging from benign to malignant. The majority of these tumors are smaller than 5 cm, whereas those larger are termed giant schwannomas. When localized in the lower legs, the maximum length of the schwannoma is less than 10 cm. We report a case of giant schwannoma of the leg and its management. CASE PRESENTATION A 11-year-old boy presented with a 13 cm × 5 cm firm, smooth, well-defined margin mass in the posterior-medial aspect of right leg. The tumor was fusiform, well capsulated, multi-lobulated soft tissue with 13 cm × 4 cm × 3 cm in size at the biggest region. On MRI the tumor was low signal, isointense with adjacent tissue on T1S, hyper-intense on T2-FS sequences and surrounded by a thin fat-like intense rim. Biopsy findings were considered most consistent with Schwannoma (Antoni A). Tumor resection was performed. The mass appeared capsulated, white, and glistening with 132 mm × 45 mm × 34 mm in size. Postoperative course was uneventful without neurological deficit. CLINICAL DISCUSSION AND CONCLUSION Schwannomas are the most common peripheral nerve sheath tumors that derived almost entirely from Schwann cells. Schwannomas usually affect the head and neck region, localization in the lower extremity is rare. When located in lower extremity, the maximum diameter of 5 cm is described in most studies. Clinical presentation of schwannomas is unclear and unspecific. Diagnosis is based on ultrasound, MRI, and histology. The recommended treatment for schwannoma is surgical enucleation or resection without damaging the involved nerve.
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Affiliation(s)
- Vinh Pham Quang
- Department of Orthopaedics and Rehabilitation, University of Medicine and Pharmacy at Ho Chi Minh City, 217 Hong Bang Street, District 5, Ho Chi Minh City, Viet Nam; Department of Orthopaedics, University Medical Center Ho Chi Minh City, 201 Nguyen Chi Thanh Street, District 5, Ho Chi Minh City, Viet Nam.
| | - Huy Hoang Quoc
- Department of Orthopaedics, University Medical Center Ho Chi Minh City, 201 Nguyen Chi Thanh Street, District 5, Ho Chi Minh City, Viet Nam.
| | - Bach Nguyen
- Department of Orthopaedics, University Medical Center Ho Chi Minh City, 201 Nguyen Chi Thanh Street, District 5, Ho Chi Minh City, Viet Nam.
| | - Chuong Ngo Quang
- Department of Orthopaedics, University Medical Center Ho Chi Minh City, 201 Nguyen Chi Thanh Street, District 5, Ho Chi Minh City, Viet Nam.
| | - Hieu Nguyen Chi
- Department of Orthopaedics, University Medical Center Ho Chi Minh City, 201 Nguyen Chi Thanh Street, District 5, Ho Chi Minh City, Viet Nam.
| | - Ngoc Nguyen
- Department of Orthopaedics, University Medical Center Ho Chi Minh City, 201 Nguyen Chi Thanh Street, District 5, Ho Chi Minh City, Viet Nam.
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22
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Istefan E, Zimmerman M, Dahlin LB, Nyman E. Benign nerve tumours in the upper limb: a registry-based study of symptoms and surgical outcome. Sci Rep 2023; 13:11500. [PMID: 37460574 DOI: 10.1038/s41598-023-38184-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2023] [Accepted: 07/04/2023] [Indexed: 07/20/2023] Open
Abstract
Surgery for benign nerve tumours is performed for pathoanatomical diagnosis and symptomatic relief, but might cause residual problems. We aimed to assess patient-reported symptoms and disability before and after surgery at a national level. In total, 206 cases surgically treated for a benign peripheral nerve tumour 2010-2019 registered in the Swedish Quality Registry for Hand Surgery (HAKIR; response rates 22-34%) were analysed. Surgery reduced overall disability in the affected limb (QuickDASH 18/100 [IQR 5-36] preoperatively and 5/100 [IQR 0-22] 12 months postoperatively), improved ability to perform daily activities (HQ-8; 11/100 [IQR 0-50] preoperatively and 0/100 [IQR 0-20] 12 months postoperatively) and decreased three evaluated pain modalities: pain at rest (HQ-8; 20/100 [IQR 0-40] preoperatively and 0/100 [IQR 0-10] 12 months postoperatively), pain on motion without load (HQ-8; 20/100 [IQR 0-40] preoperatively and 0/100 [IQR 0-10] 12 months postoperatively), and pain on load (HQ-8; 24/100 [IQR 1-69] preoperatively and 1/100 [IQR 0-30] 12 months postoperatively). Cold sensitivity was a minor problem both before and after surgery (HQ-8; 0/100 [IQR 0-30] preoperatively and 1/100 [IQR 0-40] 12 months postoperatively). We conclude that surgery for benign peripheral nerve tumours provides good symptomatic relief with low risk for residual problems.
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Affiliation(s)
- Emanuel Istefan
- Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
| | - Malin Zimmerman
- Department of Orthopaedics, Helsingborg Hospital, Helsingborg, Sweden
- Department of Translational Medicine - Hand Surgery, Lund University, Malmö, Sweden
| | - Lars B Dahlin
- Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
- Department of Translational Medicine - Hand Surgery, Lund University, Malmö, Sweden
- Department of Hand Surgery, Skåne University Hospital, Malmö, Sweden
| | - Erika Nyman
- Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden.
- Department of Hand Surgery, Plastic Surgery and Burns, Department of Biomedical and Clinical Sciences, Linköping University Hospital, 581 83, Linköping, Sweden.
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23
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Benato A, D'Alessandris QG, Murazio M, Pacelli F, Mattogno PP, Fernández E, Lauretti L. Integrated Neurosurgical Management of Retroperitoneal Benign Nerve Sheath Tumors. Cancers (Basel) 2023; 15:3138. [PMID: 37370749 DOI: 10.3390/cancers15123138] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2023] [Revised: 05/24/2023] [Accepted: 06/09/2023] [Indexed: 06/29/2023] Open
Abstract
Peripheral nerve sheath tumors (PNST) of the retroperitoneum are rare and are often treated by general surgeons dealing with retroperitoneal cancers. However, resection without the correct microsurgical technique can cause permanent neurological deficits and pain. Here, we discuss our interdisciplinary approach based on the integration of expertise from neurosurgery and abdominal surgery, allowing for both safe exposure and nerve-sparing microsurgical resection of these lesions. We present a series of 15 patients who underwent resection of benign retroperitoneal or pelvic PNST at our institution. The mean age of patients was 48.4 years; 67% were female. Tumors were 14 schwannomas and 1 neurofibroma. Eight patients (53%) reported neurologic symptoms preoperatively. The rate of complete resection was 87% (n = 13); all symptomatic patients showed improvement of their preoperative symptoms. There were no postoperative motor deficits; one patient (7%) developed a permanent sensory deficit. At a mean postoperative follow-up of 31 months, we observed no recurrences. To our best knowledge, this is the second-largest series of benign retroperitoneal PNST consistently managed with microsurgical techniques. Our experience confirms that interdisciplinary management allows for safe treatment of these tumors with good neurological and oncological outcomes.
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Affiliation(s)
- Alberto Benato
- Rome Campus, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
- Department of Neurosurgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy
| | | | - Marino Murazio
- Rome Campus, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
- Department of Surgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy
| | - Fabio Pacelli
- Rome Campus, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
- Department of Surgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy
| | - Pier Paolo Mattogno
- Department of Neurosurgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy
| | - Eduardo Fernández
- Rome Campus, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
- Department of Neurosurgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy
| | - Liverana Lauretti
- Rome Campus, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
- Department of Neurosurgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy
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Pisapia JM, Adeclat G, Roberts S, Li YR, Ali Z, Heuer GG, Zager EL. Tumors of the brachial plexus region: A 15-year experience with emphasis on motor and pain outcomes and literature review. Surg Neurol Int 2023; 14:162. [PMID: 37292396 PMCID: PMC10246342 DOI: 10.25259/sni_163_2023] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2023] [Accepted: 04/12/2023] [Indexed: 06/10/2023] Open
Abstract
Background Brachial plexus region tumors are rare. In this study, we reviewed our experience with resection of tumors involving or adjacent to the brachial plexus to identify patterns in presentation and outcome. Methods We report a retrospective case series of brachial plexus tumors operated on by a single surgeon at a single institution over 15 years. Outcome data were recorded from the most recent follow-up office visit. Findings were compared to a prior internal series and comparable series in the literature. Results From 2001 to 2016, 103 consecutive brachial plexus tumors in 98 patients met inclusion criteria. Ninety percent of patients presented with a palpable mass, and 81% had deficits in sensation, motor function, or both. Mean follow-up time was 10 months. Serious complications were infrequent. For patients with a preoperative motor deficit, the rate of postoperative motor decline was 10%. For patients without a preoperative motor deficit, the rate of postoperative motor decline was 35%, which decreased to 27% at 6 months. There were no differences in motor outcome based on extent of resection, tumor pathology, or age. Conclusion We present one of the largest recent series of tumors of the brachial plexus region. Although the rate of worsened postoperative motor function was higher in those without preoperative weakness, the motor deficit improves over time and is no worse than antigravity strength in the majority of cases. Our findings help guide patient counseling in regard to postoperative motor function.
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Affiliation(s)
- Jared M. Pisapia
- Department of Neurosurgery, Westchester Medical Center, Valhalla, New York
| | - Giscard Adeclat
- Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Sanford Roberts
- Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Yun R. Li
- Department of Radiation Oncology, University of California San Francisco, San Francisco, California
| | - Zarina Ali
- Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Gregory G. Heuer
- Department of Neurosurgery, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Eric L. Zager
- Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania
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Shekouhi R, Chim H. Patient demographics, tumor characteristics, and outcomes following surgical treatment of benign and malignant brachial plexus tumors: a systematic review. Int J Surg 2023; 109:972-981. [PMID: 37097618 PMCID: PMC10389507 DOI: 10.1097/js9.0000000000000309] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2022] [Accepted: 02/15/2023] [Indexed: 04/26/2023]
Abstract
BACKGROUND Various treatment options have been introduced for the management of primary tumors of the brachial plexus (BP), ranging from conservative therapy to wide local excision with/without postoperative chemoradiotherapy. However, no consensus exists regarding optimal treatment strategies based on collated and published data. OBJECTIVE The aim of this study was to investigate the clinicopathological characteristics and outcome of patients with primary tumors of the BP who underwent surgical treatment. DATA SOURCES A systematic search of the four main online databases, including Web of Science (WOS), PubMed, Scopus, and Google Scholar, was conducted. STUDY SELECTION All related articles addressing the clinical outcome and role of surgical interventions for management of primary tumors of the BP. INTERVENTION Optimal surgical and radiotherapeutic interventions for benign and malignant lesions based on the pathologic characteristics and location of primary BP tumors. RESULTS A total of 687 patients (693 tumors) with a mean age of 41.7±8.7 years old were evaluated. In total, 629 (90.8%) tumors were benign, and 64 (9.2%) were malignant, with a mean tumor size of 5.4±3.1 cm. The location of the tumor was reported for 639 patients. For these tumors, 444 (69.5%) originated from the supraclavicular region, and 195 (30.5%) were infraclavicular. The trunks were the most common location for tumor involvement, followed by the roots, cords, and terminal branches. Gross total resection was achieved in 432 patients and subtotal resection (STR) was performed in 109 patients. With neurofibromas, STR still resulted in good outcomes. The outcomes following treatment of malignant peripheral nerve sheath tumors were poor regardless of the type of resection. In general, symptoms related to pain and sensory issues resolved rapidly postoperatively. However, the resolution of motor deficits was often incomplete. Local tumor recurrence occurred in 15 (2.2%), patients and distant metastasis was observed in only eight (1.2%) cases. The overall mortality was 21 (3.1%) patients among the study population. LIMITATIONS The main limitation was the lack of level I and II evidence. CONCLUSIONS The ideal management strategy for primary BP tumors is complete surgical resection. However, in some cases, particularly for neurofibromas, STR may be preferable to preserve maximal neurological function. The degree of surgical excision (total or subtotal) mainly depends on the pathological characteristics and primary location of the tumor.
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Affiliation(s)
- Ramin Shekouhi
- Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Florida
| | - Harvey Chim
- Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Florida
- Lilian S. Wells Department of Neurosurgery, University of Florida College of Medicine, Gainesville, Florida, USA
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Qu Y, Yang H, Wei L, Su G. Ultrasound-guided brachial plexus nerve block in a patient with a left palmar schwannoma: A case report. Medicine (Baltimore) 2023; 102:e33440. [PMID: 37000059 PMCID: PMC10063262 DOI: 10.1097/md.0000000000033440] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/22/2023] [Accepted: 03/14/2023] [Indexed: 04/01/2023] Open
Abstract
RATIONALE Ultrasound-guided brachial plexus block is a common anesthetic procedure used in upper extremity surgery. However, it may not be a suitable option for some patients. PATIENT CONCERNS A 17-year-old woman with the left palmar schwannoma scheduled for surgical treatment received ultrasound-guided brachial plexus block. The anesthesia modalities of the disease were discussed. DIAGNOSES Based on the patient's complaints and clinical appearance, provisional diagnosis of neurofibroma was considered. INTERVENTIONS In this case, we present a case of ultrasound-guided axillary brachial plexus block used for upper extremity surgery in this patient. It was not easily and painlessly reduced in the surgery, although the visual analogue scale score was 0 and no motor movements of the left arm and palm were observed. The pain was relieved by intravenous injection of 50 mcg remifentanil. OUTCOMES Immunohistochemically labeled pathological examination confirmed the mass to be a schwannoma. There was no need to apply additional analgesia after surgery, although the patient felt numbness in the left thumb for 3 days follow up. LESSONS Even if there is painless when skin-cutting after implementation of brachial plexus block, the patient is painful when pulls the nerve around the tumor during excision. It is necessary to give an analgesic drug or anesthetize a single terminal nerve as a supplement for brachial plexus block in patients with schwannoma.
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Affiliation(s)
- Yan Qu
- Kunming Medical University, Kunming, Yunnan, P. R. China
- Department of Anesthesiology, Affiliated Hospital of Yunnan University, Kunming, Yunnan, P. R. China
| | - Haomin Yang
- Department of Anesthesiology, Affiliated Hospital of Yunnan University, Kunming, Yunnan, P. R. China
| | - Lichun Wei
- Department of Anesthesiology, Infectious Disease Hospital of Lijiang City, Lijiang, Yunnan, P. R. China
| | - Guoning Su
- Department of Anesthesiology, Affiliated Hospital of Yunnan University, Kunming, Yunnan, P. R. China
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Taka M, Kobayashi S, Mizutomi K, Inoue D, Takamatsu S, Gabata T, Matsumoto I, Ikeda H, Kobayashi T, Minato H, Abo H. Diagnostic approach for mediastinal masses with radiopathological correlation. Eur J Radiol 2023; 162:110767. [PMID: 36921376 DOI: 10.1016/j.ejrad.2023.110767] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2022] [Revised: 02/13/2023] [Accepted: 03/06/2023] [Indexed: 03/13/2023]
Abstract
PURPOSE Mediastinal masses have various histopathological and radiological findings. Although lymphoma is the most common type of tumor, thymic epithelial and neurogenic tumors are common in adults and children, respectively, but several other types are difficult to distinguish. No previous review has simply and clearly shown how to differentiate mediastinal masses. METHOD We conducted a review of the latest mediastinal classifications and mass differentiation methods, with a focus on neoplastic lesions. Both older and recent studies were searched, and imaging and histopathological findings of mediastinal masses were reviewed. Original simple-to-use differentiation flowcharts are presented. RESULTS Assessing localizations and internal characteristics is very important for mediastinal mass differentiation. The mass location and affected organ/tissue should be accurately assessed first, followed by more qualitative diagnosis, and optimization of the treatment strategy. In 2014, the International Thymic Malignancy Interest Group presented a new mediastinal clinical classification. In this classification, mediastinal masses are categorized into three groups according to location: prevascular (anterior)-, visceral (middle)-, and paravertebral (posterior)-compartment masses. Then, the internal characteristics and functional images are evaluated. CONCLUSIONS Differentiation of mediastinal masses is very difficult. However, if typical imaging findings and clinical characteristics are combined, reasonable differentiation is possible. In each patient, proper differential diagnosis may contribute to better treatment selection.
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Affiliation(s)
- Masashi Taka
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Satoshi Kobayashi
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Kaori Mizutomi
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Dai Inoue
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Shigeyuki Takamatsu
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Toshifumi Gabata
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Isao Matsumoto
- Department of Thoracic Surgery, Kanazawa University, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Hiroko Ikeda
- Department of Pathology, Kanazawa University Graduate School of Medical Sciences, 13-1, Takara-machi, Kanazawa City, Ishikawa 920-8530, Japan.
| | - Takeshi Kobayashi
- Department of Radiology, Ishikawa Prefectural Central Hospital, 2-1, Kuratsukihigashi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Hiroshi Minato
- Department of Diagnostic Pathology, Ishikawa Prefectural Central Hospital, 2-1, Kuratsukihigashi, Kanazawa City, Ishikawa 920-8641, Japan.
| | - Hitoshi Abo
- Department of Radiology, Toyama Prefectural Central Hospital, 2-2-78, Nishinagae, Toyama City, Toyama 930-8550, Japan.
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Liu Y, Li J. Metastatic adenocarcinoma of radial nerve. Am J Med Sci 2023; 365:e35-e36. [PMID: 36162453 DOI: 10.1016/j.amjms.2022.09.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2021] [Revised: 06/21/2022] [Accepted: 09/19/2022] [Indexed: 11/28/2022]
Affiliation(s)
- Ying Liu
- Department of Hand and Foot Surgery, Binzhou Medical University Hospital, Binzhou, Shandong, China
| | - Jun Li
- Department of Radiology, Yantai Affiliated Hospital of Binzhou Medical University, Yantai, Shandong, China.
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29
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Cherraqi A, El Haddad S, Messaoud O, Andour H, Tbouda M, El Ansari N, KILI A, Hessissen L, Allali N, Chat L. Saphenous Nerve Schwannoma: A Rare Differential Diagnosis of Knee Pain in Children. Glob Pediatr Health 2023; 10:2333794X231156047. [PMID: 36814534 PMCID: PMC9940164 DOI: 10.1177/2333794x231156047] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2022] [Accepted: 01/20/2023] [Indexed: 02/19/2023] Open
Abstract
Schwannomas are uncommon benign tumors of the peripheral nerves with a low risk of malignant transformation. They rarely affect children, can affect any part of the body but rarely occur in the lower extremity and typically present with a palpable mass, pain or neurological signs. Imaging helps to orient the diagnosis and anatomopathological examination helps to confirm it. We report a case of a 12-year-old girl who presented with left knee pain with subcutaneous mass overlying the tibial tuberosity medially. Clinical examination revealed a positive Tinel's sign. Magnetic resonance imaging (MRI) of the knee was performed, which revealed an encapsulated subcutaneous soft tissue mass overlying the tibial tuberosity medially, eccentric to the course of the infrapatellar branch of the saphenous nerve. The patient was operated with total intracapsular excision of the lesion and the anatomopathological study of the surgical specimen came back in favor of a schwannoma. Postoperatively, the patient showed a good recovery with disappearance of pain and swelling.
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Affiliation(s)
- Amine Cherraqi
- Mother and Child Hospital IBN SINA, Rabat, Morocco,Amine Cherraqi, Ibn Sina University Hospital Center, Rabat 10000, Morocco.
| | | | - Ola Messaoud
- Mother and Child Hospital IBN SINA, Rabat, Morocco
| | - Hajar Andour
- Mother and Child Hospital IBN SINA, Rabat, Morocco
| | | | | | | | | | - Nazik Allali
- Mother and Child Hospital IBN SINA, Rabat, Morocco
| | - Latifa Chat
- Mother and Child Hospital IBN SINA, Rabat, Morocco
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30
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Bhandarkar AR, Spinner RJ. Commentary: Surgical Management of Peripheral Nerve Pathology in Patients With Neurofibromatosis Type 2. Neurosurgery 2023; 92:e15-e16. [PMID: 36377908 DOI: 10.1227/neu.0000000000002241] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2022] [Accepted: 09/17/2022] [Indexed: 11/16/2022] Open
Affiliation(s)
- Archis R Bhandarkar
- Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.,Alix School of Medicine, Mayo Clinic, Rochester, Minnesota, USA
| | - Robert J Spinner
- Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA
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31
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Nazzi V, Innocenti N, Castelli N, Tramacere I, Eoli M, Cojazzi V, Gatti L, Acerbi F, Falco J, Vetrano IG. Assessing the role of sodium fluorescein in peripheral nerve sheath tumors and mimicking lesions surgery: An update after 142 cases. Front Oncol 2023; 12:1070878. [PMID: 36698394 PMCID: PMC9868923 DOI: 10.3389/fonc.2022.1070878] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2022] [Accepted: 12/12/2022] [Indexed: 01/12/2023] Open
Abstract
Objective Peripheral nerve sheath tumors (PNST) include mainly schwannomas and neurofibromas. Surgical resection represents the mainstay of treatment but due to their pathogenesis, distinguishing between intact functional nerve and the fibers from whence the PNST arose may not always be easy to perform, constituting the most relevant risk factor in determining a worsening in neurological condition. The introduction of intraoperative tools to better visualize these tumors could help achieve a gross-total resection. In this study, we analyzed the effect of sodium fluorescein (SF) on the visualization and resection of a large cohort of PNST. Methods Between September 2018 and December 2021, 142 consecutive patients harboring a suspected PNST underwent fluorescein-guided surgery at the Department of Neurosurgery of the Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. All patients presented with a different degree of contrast enhancement at preoperative MRI. SF was intravenously injected after intubation at 1 mg/kg. Intraoperative fluorescein characteristics and postoperative neurological and radiological outcomes were collected, analyzed, and retrospectively compared with a historical series. Results 142 patients were included (42 syndromic and 100 sporadic); schwannoma was the predominant histology, followed by neurofibroma (17 neurofibroma e 12 plexiform neurofibroma) and MPNST. Bright fluorescence was present in all cases of schwannomas and neurofibromas, although with a less homogeneous pattern, whereas it was significantly less evident for malignant PNST; perineurioma and hybrid nerve sheath tumors were characterized by a faint fluorescence enhancement. The surgical resection rate in the general population and even among the subgroups was about 66.7%; from the comparative analysis, we found a consistently higher rate of complete tumor removal in plexiform neurofibromas, 66% in the "fluorescent" group vs 44% in the "historical" group (p-value < 0.05). The rate of complications and mean surgical time were superimposable among the two populations. Conclusions SF is a valuable method for safe fluorescence-guided PNST and mimicking lesions resection. Our data showed a positive effect of fluorescein-guided surgery in increasing the rate of surgical resection of plexiform neurofibromas, suggesting a possible role in improving the functional and oncological outcome of these lesions.
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Affiliation(s)
- Vittoria Nazzi
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Niccolò Innocenti
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Nicolò Castelli
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Irene Tramacere
- Department of Research and Clinical Development, Scientific Directorate, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Marica Eoli
- Molecular Neuro-Oncology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Vittoria Cojazzi
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Laura Gatti
- Neurobiology Laboratory, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Francesco Acerbi
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Jacopo Falco
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Ignazio G. Vetrano
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy,Neurobiology Laboratory, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy,Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy,*Correspondence: Ignazio G. Vetrano,
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32
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Gagliardi F, Giordano L, Medone M, Snider S, Roncelli F, Pompeo E, Mortini P. The carotid sheath route: An option to approach retrocarotid prespinal tumors with paravertebral extension. JOURNAL OF CRANIOVERTEBRAL JUNCTION AND SPINE 2023; 14:11-15. [PMID: 37213570 PMCID: PMC10198208 DOI: 10.4103/jcvjs.jcvjs_148_22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2022] [Accepted: 12/04/2022] [Indexed: 03/16/2023] Open
Abstract
Background The elective route to approach paravertebral lesions growing into the anterolateral lodge of the neck is widely recognized as the prespinal route with its two major variants. Recently, attention has been focused on the possibility of opening the inter-carotid-jugular window in case of reparative surgery for traumatic brachial plexus injury. Aims For the first time, the authors validate the clinical application of the carotid sheath route in the surgical treatment of paravertebral lesions expanding into the anterolateral lodge of the neck. Methods A microanatomic study was conducted to collect anthropometric measurements. The technique was illustrated in a clinical setting. Results The opening of the inter-carotid-jugular surgical window allows additional access to the prevertebral and periforaminal space. It optimizes the operability on the prevertebral compartment, compared to the retro-sternocleidomastoid (SCM) approach, and on the periforaminal compartment, compared to the standard pre-SCM approach. The surgical control of the vertebral artery is comparable to that obtained with the retro-SCM approach, while the control of the esophagotracheal complex and the retroesophageal space is comparable to the pre-SCM approach. The risk profile on the inferior thyroid vessels, recurrent nerve, and sympathetic chain is superimposable to the pre-SCM approach. Conclusions The carotid sheath route is a safe and effective option to approach prespinal lesions with retrocarotid monolateral paravertebral extension.
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Affiliation(s)
- Filippo Gagliardi
- Department of Neurosurgery and Gamma Knife Radiosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy
| | - Leone Giordano
- Department of Otorhinolaryngology, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy
| | - Marzia Medone
- Department of Neurosurgery and Gamma Knife Radiosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy
| | - Silvia Snider
- Department of Neurosurgery and Gamma Knife Radiosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy
| | - Francesca Roncelli
- Department of Neurosurgery and Gamma Knife Radiosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy
| | - Edoardo Pompeo
- Department of Neurosurgery and Gamma Knife Radiosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy
| | - Pietro Mortini
- Department of Neurosurgery and Gamma Knife Radiosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy
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Dengler NF, Scholz C, Beck J, Uerschels AK, Sure U, Scheller C, Strauss C, Martin D, Schackert G, Heinen C, Woitzik J, McLean AL, Rosahl SK, Kolbenschlag J, Heinzel J, Schuhmann M, Tatagiba MS, Guerra WKW, Schroeder HWS, Vetrano IG, Ahmadi R, Unterberg A, Reinsch J, Zdunczyk A, Unteroberdoerster M, Vajkoczy P, Wehner S, Becker M, Matthies C, Pérez-Tejón J, Dubuisson A, Barrone DG, Trivedi R, Capone C, Ferraresi S, Kraschl J, Kretschmer T, Dombert T, Staub F, Ronellenfitsch M, Marquardt G, Prinz V, Czabanka M, Carolus A, Braun V, König R, Antoniadis G, Wirtz CR, Rasulic L, Pedro MT. Rationale and design of the peripheral nerve tumor registry: an observational cohort study. Neurol Res 2023; 45:81-85. [PMID: 36208460 DOI: 10.1080/01616412.2022.2129762] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/14/2023]
Abstract
AIM Peripheral nerve tumors (PNT) are rare lesions. To date, no systematic multicenter studies on epidemiology, clinical symptoms, treatment strategies and outcomes, genetic and histopathologic features, as well as imaging characteristics of PNT were published. The main goal of our PNT Registry is the systematic multicenter investigation to improve our understanding of PNT and to assist future interventional studies in establishing hypotheses, determining potential endpoints, and assessing treatment efficacy. METHODS Aims of the PNT registry were set at the 2015 Meeting of the Section of Peripheral Nerve Surgery of the German Society of Neurosurgery. A study protocol was developed by specialists in PNT care. A minimal data set on clinical status, treatment types and outcomes is reported by each participating center at initial contact with the patient and after 1 year, 2 years, and 5 years. Since the study is coordinated by the Charité Berlin, the PNR Registry was approved by the Charité ethics committee (EA4/058/17) and registered with the German Trials Registry (www.drks.de). On a national level, patient inclusion began in June 2016. The registry was rolled out across Europe at the 2019 meeting of the European Association of Neurosurgery in Dublin. RESULTS Patient recruitment has been initiated at 10 centers throughout Europe and 14 additional centers are currently applying for local ethics approval. CONCLUSION To date, the PNT registry has grown into an international study group with regular scientific and clinical exchange awaiting the first results of the retrospective study arm.
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Affiliation(s)
- Nora F Dengler
- Department of Neurosurgery, Charité - Universitaetsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
| | - Christoph Scholz
- Department of Neurosurgery, Medical Center, Faculty of Medicine, University of Freiburg, Hugstetter Str. 55, 79106 Freiburg i.B, Germany
| | - Jürgen Beck
- Department of Neurosurgery, Medical Center, Faculty of Medicine, University of Freiburg, Hugstetter Str. 55, 79106 Freiburg i.B, Germany
| | - Anne-Kathrin Uerschels
- Department of Neurosurgery, University Hospital Essen, University of Duisburg-Essen, Hufelandstrasse 55, 45147 Essen, Germany
| | - Ullrich Sure
- Department of Neurosurgery, University Hospital Essen, University of Duisburg-Essen, Hufelandstrasse 55, 45147 Essen, Germany
| | - Christian Scheller
- Department of Neurosurgery, University of Halle, Ernst-Grube-Str. 40, 06120 Halle, Germany
| | - Christian Strauss
- Department of Neurosurgery, University of Halle, Ernst-Grube-Str. 40, 06120 Halle, Germany
| | - Daniel Martin
- Department of Neurosurgery, Technische Universität Dresden, Fetscherstr. 74, 01307 Dresden, Germany
| | - Gabriele Schackert
- Department of Neurosurgery, Technische Universität Dresden, Fetscherstr. 74, 01307 Dresden, Germany
| | - Christian Heinen
- Department of Neurosurgery, Evangelisches Krankenhaus, Carl von Ossietzky University Oldenburg, Marienstr. 11, 26121 Oldenburg, Germany.,Department of Neurosurgery, PeripheralNerveUnit Nord, Christliches Krankenhaus Quakenbrück GmbH, Quakenbrück, Germany
| | - Johannes Woitzik
- Department of Neurosurgery, Evangelisches Krankenhaus, Carl von Ossietzky University Oldenburg, Marienstr. 11, 26121 Oldenburg, Germany
| | - Anna Lawson McLean
- Department of Neurosurgery, HELIOS Klinikum Erfurt, Nordhäuser Str. 74, 99089 Erfurt, Germany
| | - Steffen K Rosahl
- Department of Neurosurgery, HELIOS Klinikum Erfurt, Nordhäuser Str. 74, 99089 Erfurt, Germany
| | - Jonas Kolbenschlag
- Department of Hand-, Plastic, Reconstructive, and Burn Surgery, BG Klinik Tübingen, Schnarrenbergstraße 95, 72076 Tübingen Germany
| | - Johannes Heinzel
- Department of Hand-, Plastic, Reconstructive, and Burn Surgery, BG Klinik Tübingen, Schnarrenbergstraße 95, 72076 Tübingen Germany
| | - Martin Schuhmann
- Department of Neurosurgery, Universitätsklinikum Tübingen, Hippe-Seyler-Straße 3, 72076 Tübingen Germany
| | - Marco Soares Tatagiba
- Department of Neurosurgery, Universitätsklinikum Tübingen, Hippe-Seyler-Straße 3, 72076 Tübingen Germany
| | | | - Henry W S Schroeder
- Department of Neurosurgery, University Medicine Greifswald, Sauerbruchstr. 1, 17475 Greifswald, Germany
| | - Ignazio Gaspare Vetrano
- Department of Neurosurgery, Fondazione I.R.C.C.S Istituto Neurologico Carlo Besta, Via Giovanni Celoria 11, 20133 Milano, Italy
| | - Rezvan Ahmadi
- Department of Neurosurgery, Heidelberg University Hospital, Berlin, Germany
| | - Andreas Unterberg
- Department of Neurosurgery, Heidelberg University Hospital, Berlin, Germany
| | - Jennifer Reinsch
- Department of Neurosurgery, Charité - Universitaetsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
| | - Anna Zdunczyk
- Department of Neurosurgery, Charité - Universitaetsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
| | - Meike Unteroberdoerster
- Department of Neurosurgery, Charité - Universitaetsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
| | - Peter Vajkoczy
- Department of Neurosurgery, Charité - Universitaetsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
| | - Sarah Wehner
- Department of Plastic, Reconstructive, and Hand Surgery, Burn Center, University Hospital RWTH Aachen, Aachen, Germany
| | - Michael Becker
- Department of Plastic, Reconstructive, and Hand Surgery, Burn Center, University Hospital RWTH Aachen, Aachen, Germany
| | - Cordula Matthies
- Department of Neurosurgery, Würzburg University Hospital, Würzburg, Germany
| | - Jose Pérez-Tejón
- Department of Neurosurgery, Würzburg University Hospital, Würzburg, Germany
| | - Annie Dubuisson
- Department of Neurosurgery, CHU Liège, Avenue de L'Hôpital 1, Liège, Belgium
| | - Damiano G Barrone
- Department of Clinical Neurosciences, University of Cambridge School of Clinical Medicine, Cambridge, UK
| | - Rikin Trivedi
- Department of Clinical Neurosciences, University of Cambridge School of Clinical Medicine, Cambridge, UK
| | - Crescenzo Capone
- Division of Neurosurgery, Department of Neurosciences, Reproductive and Odontostomatological Sciences, School of Medicine and Surgery, University Napoli "Frederico II", Naples, Italy
| | - Stefano Ferraresi
- Department of Neurosurgery, Ospedale Santa Maria della Misericordia, Rovigo, Italy
| | - Jakob Kraschl
- Department of Neurosurgery and Neurorestoration, Klinikum Klagenfurt Am Wörthersee, Klagenfurt, Austria
| | - Thomas Kretschmer
- Department of Neurosurgery and Neurorestoration, Klinikum Klagenfurt Am Wörthersee, Klagenfurt, Austria
| | | | - Frank Staub
- Center for Peripheral Neurosurgery, Dossenheim, Germany
| | - Michael Ronellenfitsch
- Dr. Senckenberg Institute of Neurooncology, University Hospital Frankfurt, Goethe University, Schleusenweg, Frankfurt am Main, Germany
| | - Gerhard Marquardt
- Department of Neurosurgery, University Hospital Frankfurt, Goethe University, Siegen, Germany
| | - Vincent Prinz
- Department of Neurosurgery, University Hospital Frankfurt, Goethe University, Siegen, Germany
| | - Marcus Czabanka
- Department of Neurosurgery, University Hospital Frankfurt, Goethe University, Siegen, Germany
| | - Anne Carolus
- Department of Neurosurgery, Diakonie Klinikum Jung-Stilling-Krankenhaus Neurochriurgische Klinik, Siegen, Germany
| | - Veit Braun
- Department of Neurosurgery, Diakonie Klinikum Jung-Stilling-Krankenhaus Neurochriurgische Klinik, Siegen, Germany
| | - Ralph König
- Peripheral Nerve Surgery Unit, Department of Neurosurgery, Ulm University, District Hospital, Günzburg, Germany
| | - Gregor Antoniadis
- Peripheral Nerve Surgery Unit, Department of Neurosurgery, Ulm University, District Hospital, Günzburg, Germany
| | - Christian Rainer Wirtz
- Peripheral Nerve Surgery Unit, Department of Neurosurgery, Ulm University, District Hospital, Günzburg, Germany
| | - Lukas Rasulic
- Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Clinic for Neurosurgery, Clinical Center of Serbia, Belgrade, Serbia
| | - Maria Teresa Pedro
- Peripheral Nerve Surgery Unit, Department of Neurosurgery, Ulm University, District Hospital, Günzburg, Germany
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Lubelski D, Pennington Z, Ochuba A, Azad TD, Mansouri A, Blakeley J, Belzberg AJ. Natural History of Brachial Plexus, Peripheral Nerve, and Spinal Schwannomas. Neurosurgery 2022; 91:883-891. [PMID: 36069570 DOI: 10.1227/neu.0000000000002118] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2021] [Accepted: 06/20/2022] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND Management of sporadic schwannomas is often dictated by a patient's clinical presentation and the tumor's behavior. For patients who are managed nonsurgically, there are little data available about the expected natural history. OBJECTIVE To evaluate the natural history and growth patterns of extracranial schwannomas including tumors of the distal peripheral nerves, spine, and brachial plexus. METHODS A retrospective review was performed to identify patients with nonsyndromic extracranial schwannomas at a single tertiary care institution diagnosed between 2002 and 2019. Patient data and tumor characteristics including volume were recorded. RESULTS Two hundred twenty-seven patients were identified (mean age 51 years, 42% male, average of 27.8-month follow-up). Tumor location was distal peripheral nerve in 82, brachial plexus in 36, and paraspinal in 109. At the time of diagnosis, peripheral lesions were significantly larger than spinal (59 m 3 vs 13 cm 3 ) and brachial plexus lesions (15 cm 3 ). Distinct growth patterns were seen with both distal peripheral nerve and spinal lesions; 34/82 peripheral nerve lesions had fast growth (β = 0.176%/day), and 48 had slow growth (β = 0.021%/day; P < .01). Spinal schwannomas similarly had 30 fast-growing (β = 0.229%/day), 16 moderate-growing (β = 0.071%/day), and 63 slow-growing (β = 0.022%/day; P = .03) subtypes. The brachial plexus had relatively homogeneous growth patterns (β = 0.065%/day). Females had 2.9 times greater odds of having the fast-growing subtype. CONCLUSION Distinct growth patterns were seen in extracranial sporadic schwannomas based on tumor location and patient demographics. Fast (>80% volume change per year) vs slow (5%-10% per year) tumor growth can often be ascertained within 2 follow-up images. Awareness of these patterns might have implications for patient counseling and therapeutic decision-making.
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Affiliation(s)
- Daniel Lubelski
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Zach Pennington
- Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA
| | - Arinze Ochuba
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Tej D Azad
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Alireza Mansouri
- Department of Neurosurgery, Penn State Health, Hershey, Pennsylvania, USA
| | - Jaishri Blakeley
- Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Allan J Belzberg
- Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
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Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1. J Clin Med 2022; 11:jcm11195695. [PMID: 36233563 PMCID: PMC9571780 DOI: 10.3390/jcm11195695] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2022] [Revised: 09/23/2022] [Accepted: 09/24/2022] [Indexed: 11/21/2022] Open
Abstract
Background: Nodular plexiform neurofibromas in individuals with neurofibromatosis type 1 often cause significant symptoms and are treated with surgical excision despite the potential risk of complications. This study aimed to clarify the surgical outcomes of deep-seated nodular plexiform neurofibromas and identify the factors associated with postoperative complications. Methods: We retrospectively reviewed patients with neurofibromatosis type 1 who underwent surgical excision for deep-seated nodular plexiform neurofibromas in our hospital from 2015 to 2021. Enucleation while preserving the nerve fascicles was attempted first, and en bloc resection, ligating the nerve origin in cases in which the parent nerve was entrapped by the tumor, making the tumor difficult to dissect, was performed. Results: In 15 patients, 24 nodular plexiform neurofibromas received surgical excision. Sixteen tumors were enucleated, and eight were en bloc resected. The symptoms of all 10 patients with preoperative symptoms resolved after surgery. Four patients developed new neurological deficits immediately after surgery, two of whom had retained neurological symptoms at the last visit, but these symptoms were mild. Conclusions: The present study demonstrates that surgical treatment of nodular plexiform neurofibromas, even deep-seated neurofibromas, is safe with a low risk of severe complications and improvement in preoperative symptoms.
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Suárez C, López F, Rodrigo JP, Mendenhall WM, de Bree R, Mäkitie AA, Vander Poorten V, Takes RP, Bondi S, Kowalski LP, Shaha AR, Fernández-Alvarez V, Gutiérrez JC, Zidar N, Chiesa-Estomba C, Strojan P, Sanabria A, Rinaldo A, Ferlito A. Benign Peripheral Non-cranial Nerve Sheath Tumors of the Neck. Adv Ther 2022; 39:3449-3471. [PMID: 35689724 DOI: 10.1007/s12325-022-02191-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/01/2022] [Accepted: 05/11/2022] [Indexed: 11/26/2022]
Abstract
Benign peripheral non-cranial nerve sheath tumors are rare lesions, including both schwannomas and neurofibromas. These tumors arise from Schwann cells, and may originate from any peripheral, cranial, or autonomic nerve. Most of them are localized and sporadic but multifocal systemic forms can occur. Cervical sympathetic chain, brachial plexus, cervical plexus and spinal roots and nerves are the major nerve systems commonly affected. Dumbbell-shaped intra- and extradural tumors occur most commonly in the cervical spine, as well as purely extradural and paravertebral tumors. The management of these tumors has improved greatly owing to the developments in imaging techniques and surgical innovations such as endoscopically assisted approaches and robotic surgery. Microsurgical intracapsular excision of the tumor helped by the use of intraoperative fluorescent dyes and intraoperative neurophysiological monitoring minimize postoperative neural deficit, since most schwannomas are encapsulated. Most tumors can be removed with a low rate of complications and recurrence. Radiotherapy should be considered for growing lesions that are not amenable to surgery. In asymptomatic patients, observation and serial scans is an option for elderly infirm patients.
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Affiliation(s)
- Carlos Suárez
- Instituto de Investigación Sanitaria del Principado de Asturias, Avenida de Roma s/n, 33011, Oviedo, Spain.
| | - Fernando López
- Instituto de Investigación Sanitaria del Principado de Asturias, Avenida de Roma s/n, 33011, Oviedo, Spain.
- Department of Otolaryngology, Hospital Universitario Central de Asturias, Instituto Universitario de Oncología del Principado de Asturias, University of Oviedo, CIBERONC, Avenida de Roma s/n, 33011, Oviedo, Spain.
| | - Juan P Rodrigo
- Instituto de Investigación Sanitaria del Principado de Asturias, Avenida de Roma s/n, 33011, Oviedo, Spain
- Department of Otolaryngology, Hospital Universitario Central de Asturias, Instituto Universitario de Oncología del Principado de Asturias, University of Oviedo, CIBERONC, Avenida de Roma s/n, 33011, Oviedo, Spain
| | - William M Mendenhall
- Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, USA
| | - Remco de Bree
- Department of Head and Neck Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands
| | - Antti A Mäkitie
- Department of Otorhinolaryngology-Head and Neck Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Vincent Vander Poorten
- Department of Oncology, Section Head and Neck Oncology, KU Leuven, Leuven, Belgium
- Otorhinolaryngology, Head and Neck Surgery, Leuven Cancer Institute, University Hospitals Leuven, Leuven, Belgium
| | - Robert P Takes
- Department of Otolaryngology-Head and Neck Surgery, Radboud University Medical Center, Nijmegen, The Netherlands
| | - Stefano Bondi
- Head and Neck Oncology, Candiolo Cancer Institute, FPO-IRCCS, Candiolo, TO, Italy
| | - Luiz P Kowalski
- Head and Neck Surgery and Otorhinolaryngology Department, A C Camargo Cancer Center and University of São Paulo Medical School, São Paulo, Brazil
| | - Ashok R Shaha
- Head and Neck Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
| | | | - Julio C Gutiérrez
- Department of Neurosurgery, Hospital Universitario Central de Asturias, Oviedo, Spain
| | - Nina Zidar
- Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Carlos Chiesa-Estomba
- Department of Otorhinolaryngology-Head and Neck Surgery, Hospital Universitario Donostia, San Sebastián, Spain
| | - Primoz Strojan
- Department of Radiation Oncology, Institute of Oncology, Ljubljana, Slovenia
| | - Alvaro Sanabria
- Department of Surgery, School of Medicine, Universidad de Antioquia/Hospital Universitario San Vicente Fundación, 050010, Medellín, Colombia
- CEXCA Centro de Excelencia en Enfermedades de Cabeza y Cuello, 050021, Medellín, Colombia
| | | | - Alfio Ferlito
- Coordinator of the International Head and Neck Scientific Group, Padua, Italy
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Hamdy O, Emile SH, Ali NM, Sabry AM, Deniwar MA. Retrospective cohort study of the characteristics and outcome of surgical treatment of pelvic neurogenic and presacral tumors. INTERDISCIPLINARY NEUROSURGERY 2022; 28:101496. [DOI: 10.1016/j.inat.2022.101496] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
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El Sayed L, H Masmejean E, Lavollé A, Biau D, Peyre M. Clinical results after surgical resection of benign solitary schwannomas: A review of 150 cases. Orthop Traumatol Surg Res 2022; 108:103281. [PMID: 35477039 DOI: 10.1016/j.otsr.2022.103281] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/14/2020] [Revised: 03/24/2021] [Accepted: 06/08/2021] [Indexed: 02/03/2023]
Abstract
INTRODUCTION Peripheral nerve schwannomas are the most common nerve tumors. While they are efficiently treated with surgery, the risk of neurological complications, especially sensory deficits, remains debated. HYPOTHESIS We postulate that rates of post-operative sensory deficits in peripheral schwannoma surgery are low and are not increased in schwannomas of the hand, in which preservation of discriminative tact is of prime functional importance. MATERIALS AND METHODS A retrospective multicenter study was carried out on 150 patients with an isolated peripheral schwannoma operated between 2002 and 2018, including 11 patients with schwannomas of the hand. All cases were reviewed preoperatively and postoperatively with a detailed exam of the neurological status. RESULTS Most schwannomas were located in the lower limbs (58%). The two main affected trunks were the posterior tibial nerve in the lower limb and the median nerve in the upper limb. The average preoperative tumor volume measured on MRI was 2.93 cm3 [0.11 cm3-25 cm3]. The most common preoperative symptoms were paresthesia (77.1%) and pseudo-Tinel sign (55.7%). Less frequently, pain (50.3%) and hypoesthesia (18.1%) were observed. On the other hand, preoperative neurological motor manifestations remained exceptional (3.3%). The rates of new post-operative motor deficits, paresthesias, and sensory deficits were 4.6%, 10.9% and 8% respectively and were not increased in schwannomas of the collateral nerves of the hand. CONCLUSION Surgical resection is a safe procedure for peripheral nerve schwannoma treatment with satisfying functional results and an acceptable risk of nerve injury, especially for sensory function. LEVEL OF PROOF D; Multicenter retrospective study.
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Affiliation(s)
- Laila El Sayed
- Hand and upper extremity surgery unit, Georges-Pompidou European Hospital (HEGP), Paris, France; University of Paris, Medical School, Paris, France.
| | - Emmanuel H Masmejean
- Hand and upper extremity surgery unit, Georges-Pompidou European Hospital (HEGP), Paris, France; University of Paris, Medical School, Paris, France; Clinique Blomet (Research Unit), Paris, France
| | | | - David Biau
- University of Paris, Medical School, Paris, France; Orthopedic Surgery, Hôpital Cochin, Paris, France
| | - Matthieu Peyre
- Genetics and Development of Brain Tumors - CRICM Inserm U1127 CNRS UMR 7225- Brain Institute - Hôpital Pitié-Salpêtrière, Paris, France
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Behzad B, Dianat S. Magnetic Resonance Imaging of Nerve Tumors. Semin Musculoskelet Radiol 2022; 26:172-181. [PMID: 35609578 DOI: 10.1055/s-0042-1742704] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
Nerve tumors are uncommon soft tissue neoplasms predominantly arising from peripheral nerve sheath and Schwann cells. We review the manifestations of benign peripheral nerve sheath tumors, concentrating on distinguishing imaging features of schwannomas versus neurofibromas with an emphasis on treatment implications. Nevertheless, there is often an overlap between the imaging presentation of these two conditions, making the accurate radiologic diagnosis challenging. Therefore, tissue sampling is often needed for a definitive histologic diagnosis. Treatment planning largely depends on symptoms, location of the lesion, and underlying risk factors. Three major syndromes, neurofibromatosis type 1, type 2, and schwannomatosis, predispose patients to peripheral nerve sheath tumors (PNSTs), with particular concern about the malignant subtype expression. In patients with suspected PNSTs, correlation of imaging findings with clinical findings and genetic tests is helpful for a more accurate diagnosis and disease management. Some imaging features on magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography can be helpful to differentiate malignant from benign subtypes.
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Affiliation(s)
- Barzin Behzad
- Department of Radiology, Division of Musculoskeletal Radiology, Penn Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Saeed Dianat
- Department of Radiology, Division of Musculoskeletal Radiology, Penn Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
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Lin Y, Li P, Chen X, Zhu J, Lu Y, Yu F, Xiao J, Wang J. Predicting the Risk of Postoperative Complications of Schwannoma Surgery: Development and Assessment of a New Predictive Nomogram. J Neurol Surg A Cent Eur Neurosurg 2022; 83:427-434. [PMID: 35537463 DOI: 10.1055/s-0041-1739500] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
BACKGROUND The aim of this study was to develop and internally validate a risk nomogram for postoperative complications of schwannoma surgery. METHODS From 2016 to 2020, we reviewed 83 patients who underwent schwannoma resection with a total number of 85 schwannomas. A predictive model was developed based on the dataset of this group. During model construction, univariate and multivariate logistic regression analysis were used to determine the independent predictors of postoperative complications. Assessment of the discriminative function, calibrating proficiency, and clinical usefulness of the predicting model was performed using C-index, calibration plot, receiver operating characteristic (ROC) curve, and decision curve analysis. Internal validation was assessed using bootstrapping validation. RESULTS Predictors contained in the prediction nomogram included age, tumor location, symptoms, and surgical approach. The model displayed satisfying abilities of discrimination and calibration, with a C-index of 0.901 (95% confidence [CI]: 0.837-0.965). A high C-index value of 0.853 was achieved in the interval verification. Decision curve analysis showed that the nomogram was clinically useful when intervention was decided at the complication possibility threshold of 2%. CONCLUSION This new risk nomogram for postoperative complications of schwannoma surgery has taken age, tumor location, symptoms, and surgical approach into account. It has reasonable predictive accuracy and can be conveniently used. It shall help patients understand the risk of postoperative complications before surgery, and offer guidance to surgeons in deciding on the surgical approach.
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Affiliation(s)
- Yutian Lin
- Department of Hand Surgery and Peripheral Neurosurgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Peifeng Li
- Department of Hand Surgery and Peripheral Neurosurgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Xiangxiang Chen
- Department of Hand Surgery and Peripheral Neurosurgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Junyi Zhu
- Department of Hand Surgery and Peripheral Neurosurgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Yingfeng Lu
- Department of Hand Surgery and Peripheral Neurosurgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Fangzheng Yu
- Department of Hand Surgery and Peripheral Neurosurgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Jian Xiao
- School of Pharmaceutical Science, Wenzhou Medical University, Wenzhou, Zhejiang, China
| | - Jian Wang
- Department of Hand Surgery and Peripheral Neurosurgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
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Morphological Relation of Peripheral Nerve Sheath Tumors and Nerve Fascicles: Prospective Study and Classification. J Clin Med 2022; 11:jcm11030552. [PMID: 35160001 PMCID: PMC8836650 DOI: 10.3390/jcm11030552] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2021] [Revised: 01/19/2022] [Accepted: 01/20/2022] [Indexed: 11/17/2022] Open
Abstract
Removal of benign peripheral nerve sheath tumors (bPNST) represents a surgical challenge. The morphological relation of bPNST and healthy nerve fascicles are of utmost importance for achieving both removal of the entire tumor and preservation of functional integrity of the peripheral nerve. Thus, we intraoperatively assessed the morphological patterns between bPNST and nerve fascicles using photo documentation obtained between January 2009 and September 2021. In 31 patients (20 women and 11 men) with a mean age of 48 ± 18 years a total of 34 bPNST were removed. Four constant morphological patterns between bPNST relatively to nerve fascicles were detected: (1) bPNST is located peripherally (n = 16), (2) it splits the nerve into two main fascicles (n = 5), (3) it totally splits up the nerve out of the nerve's center (n = 8) und (4) it encloses the nerve and its fascicles (n = 5) without any detectable boundary layer. Histology revealed 28 schwannomas, five neurofibromas, and one perineurioma. The proposed classification reflects the increasing complexity of tumor removal with a higher type number. This might be beneficial for preoperative diagnostics, i.e., high-resolution ultrasound or MRI-tractography, as well as for planning the bPNST's surgical resection and the possible need for nerve reconstruction.
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Geitenbeek RTJ, Martin E, Graven LH, Broen MPG, Anten MHME, van der Pol JAJ, Verhoef C, Taal W. Diagnostic value of 18F-FDG PET-CT in detecting malignant peripheral nerve sheath tumors among adult and pediatric neurofibromatosis type 1 patients. J Neurooncol 2022; 156:559-567. [PMID: 35025020 PMCID: PMC8860956 DOI: 10.1007/s11060-021-03936-y] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2021] [Accepted: 12/24/2021] [Indexed: 11/26/2022]
Abstract
Purpose Detecting malignant peripheral nerve sheath tumors (MPNSTs) remains difficult. 18F-FDG PET-CT has been shown helpful, but ideal threshold values of semi-quantitative markers remain unclear, partially because of variation among scanners. Using EU-certified scanners diagnostic accuracy of ideal and commonly used 18F-FDG PET-CT thresholds were investigated and differences between adult and pediatric lesions were evaluated. Methods A retrospective cohort study was performed including patients from two hospitals with a clinical or radiological suspicion of MPNST between 2013 and 2019. Several markers were studied for ideal threshold values and differences among adults and children. A diagnostic algorithm was subsequently developed. Results Sixty patients were included (10 MPNSTs). Ideal threshold values were 5.8 for SUVmax (sensitivity 0.70, specificity 0.92), 5.0 for SUVpeak (sensitivity 0.70, specificity 0.97), 1.7 for TLmax (sensitivity 0.90, specificity 0.86), and 2.3 for TLmean (sensitivity 0.90, specificity 0.79). The standard TLmean threshold value of 2.0 yielded a sensitivity of 0.90 and specificity of 0.74, while the standard SUVmax threshold value of 3.5 yielded a sensitivity of 0.80 and specificity of 0.63. SUVmax and adjusted SUV for lean body mass (SUL) were lower in children, but tumor-to-liver ratios were similar in adult and pediatric lesions. Using TLmean > 2.0 or TLmean < 2.0 and SUVmax > 3.5, a sensitivity and specificity of 1.00 and 0.63 can be achieved. Conclusion 18F-FDG PET-CT offers adequate accuracy to detect MPNSTs. SUV values in pediatric MPNSTs may be lower, but tumor-to-liver ratios are not. By combining TLmean and SUVmax values, a 100% sensitivity can be achieved with acceptable specificity. Supplementary Information The online version contains supplementary material available at 10.1007/s11060-021-03936-y.
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Affiliation(s)
- Ritch T J Geitenbeek
- Department of Plastic and Reconstructive Surgery G04.126, University Medical Center Utrecht, PO Box 85060, 3508 AB, Utrecht, The Netherlands.,Department of Surgical Oncology, Erasmus Medical Center Cancer Institute, Rotterdam, Netherlands
| | - Enrico Martin
- Department of Plastic and Reconstructive Surgery G04.126, University Medical Center Utrecht, PO Box 85060, 3508 AB, Utrecht, The Netherlands. .,Department of Surgical Oncology, Erasmus Medical Center Cancer Institute, Rotterdam, Netherlands.
| | - Laura H Graven
- Department of Radiology and Nuclear Medicine, Erasmus Medical Center Cancer Institute, Rotterdam, Netherlands
| | - Martijn P G Broen
- Department of Neurology, Maastricht University Medical Center, Maastricht, Netherlands
| | - Monique H M E Anten
- Department of Neurology, Maastricht University Medical Center, Maastricht, Netherlands
| | - Jochem A J van der Pol
- Department of Radiology and Nuclear Medicine, Maastricht University Medical Center, Maastricht, Netherlands
| | - Cornelis Verhoef
- Department of Surgical Oncology, Erasmus Medical Center Cancer Institute, Rotterdam, Netherlands
| | - Walter Taal
- Department of Neurology, Erasmus Medical Center Cancer Institute, Rotterdam, Netherlands
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El Ghazoui A, Allaoui M, El Asraoui L, Zaddoug O. Unusual presentation of lateral sural cutaneous nerve schwannoma: An exceptional case report. Int J Surg Case Rep 2022; 90:106603. [PMID: 34973628 PMCID: PMC8728400 DOI: 10.1016/j.ijscr.2021.106603] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2021] [Revised: 11/09/2021] [Accepted: 11/09/2021] [Indexed: 11/13/2022] Open
Abstract
Introduction Schwannoma is the most common benign nerve sheath tumor. Peripheral nerves of the lower extremity are rarely involved and usually asymptomatic. Case presentation We report the case of a misleading clinical presentation of lateral sural cutaneous nerve schwannoma. Discussion To the best of our knowledge, no case has been reported about the location of schwannoma in the lateral sural cutaneous nerve. MRI and anatomopathologic assessment, after microscopic enucleation, are required to confirm diagnosis. Conclusion Care must be taken to not miss a schwannoma of lateral sural cutaneous nerve by meticulous clinical examination and appropriate imaging using MRI in unexplained L5 sciatica.
Schwannoma is the most common benign nerve sheath tumor Peripheral nerves of the lower extremity are rarely involved and usually asymptomatic MRI shows isointense or decreased signal relative to the skeletal muscle on T1-weighted images and heterogeneously increased signal intensity on T2-weighted images Microscopic enucleation is treatment of choice
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Affiliation(s)
- A El Ghazoui
- Department of Orthopedics and Trauma Surgery "I", Military Hospital of Instruction Mohammed V, FAR Avenue, 10100 Rabat, Morocco.
| | - M Allaoui
- Department of Anatomopathology, Military Hospital of Instruction Mohammed V, FAR Avenue, 10100 Rabat, Morocco
| | - L El Asraoui
- Department Nuclear Medicine, Military Hospital of Instruction Mohammed V, FAR Avenue, 10100 Rabat, Morocco
| | - O Zaddoug
- Department of Orthopedics and Trauma Surgery "I", Military Hospital of Instruction Mohammed V, FAR Avenue, 10100 Rabat, Morocco
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Strommen JA, Skinner S, Crum BA. Neurophysiology during peripheral nerve surgery. HANDBOOK OF CLINICAL NEUROLOGY 2022; 186:295-318. [PMID: 35772892 DOI: 10.1016/b978-0-12-819826-1.00022-3] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/15/2023]
Abstract
Electrophysiological monitoring of the peripheral nervous system during a variety of surgeries provides useful information that supplements and complements preoperative assessment. Monitoring improves localization and understanding of the underlying pathophysiology of peripheral nerve lesions leading to more rational treatment decisions and improved outcomes. Monitoring is accomplished by adaptation of routine electrodiagnostic techniques (i.e., nerve conduction studies, evoked potentials, and electromyography) with special attention to technical factors including electrical and movement artifact. These techniques have been successfully applied during surgery for entrapment neuropathies, traumatic nerve injury and repair, peripheral nerve tumors, and adjacent structure procedures that risk peripheral nerve injury. A clear understanding of the anatomy and neurophysiology is necessary, as is understanding and performing the difficult technical aspects of these studies to provide accurate information to enhance patient outcome and recovery. As in any intraoperative neurophysiologic monitoring (IONM) setting, constant and accurate communication between the IONM team, surgeon, and anesthesia team is critically important to meet these goals.
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Affiliation(s)
- Jeffrey A Strommen
- Department of Neurophysiology, Abbott Northwestern Hospital, Minneapolis, MN, United States.
| | - Stanley Skinner
- Department of Intraoperative Neurophysiology, Abbott Northwestern Hospital, Minneapolis, MN, United States.
| | - Brian A Crum
- Department of Neurology, Mayo Clinic, Rochester, MN, United States
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Vetrano IG, Dei Cas M, Nazzi V, Eoli M, Innocenti N, Saletti V, Potenza A, Carrozzini T, Pollaci G, Gorla G, Paroni R, Ghidoni R, Gatti L. The Lipid Asset Is Unbalanced in Peripheral Nerve Sheath Tumors. Int J Mol Sci 2021; 23:ijms23010061. [PMID: 35008487 PMCID: PMC8744637 DOI: 10.3390/ijms23010061] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2021] [Revised: 12/19/2021] [Accepted: 12/20/2021] [Indexed: 12/11/2022] Open
Abstract
Peripheral nerve sheath tumors (PNSTs) include schwannomas, neurofibromas (NFs), and plexiform neurofibromas (PNFs), among others. While they are benign tumors, according to their biological behavior, some have the potential for malignant degeneration, mainly PNFs. The specific factors contributing to the more aggressive behavior of some PNSTs compared to others are not precisely known. Considering that lipid homeostasis plays a crucial role in fibrotic/inflammatory processes and in several cancers, we hypothesized that the lipid asset was also unbalanced in this group of nerve tumors. Through untargeted lipidomics, NFs presented a significant increase in ceramide, phosphatidylcholine, and Vitamin A ester. PNFs displayed a marked decrease in 34 out of 50 lipid class analyzed. An increased level of ether- and oxidized-triacylglycerols was observed; phosphatidylcholines were reduced. After sphingolipidomic analysis, we observed six sphingolipid classes. Ceramide and dihydroceramides were statistically increased in NFs. All the glycosylated species appeared reduced in NFs, but increased in PNFs. Our findings suggested that different subtypes of PNSTs presented a specific modulation in the lipidic profile. The untargeted and targeted lipidomic approaches, which were not applied until now, contribute to better clarifying bioactive lipid roles in PNS natural history to highlight disease molecular features and pathogenesis.
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Affiliation(s)
- Ignazio G. Vetrano
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (V.N.); (N.I.)
- Correspondence:
| | - Michele Dei Cas
- Department of Health Sciences, Università degli Studi di Milano, 20142 Milan, Italy; (M.D.C.); (R.P.)
| | - Vittoria Nazzi
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (V.N.); (N.I.)
| | - Marica Eoli
- Molecular Neuro-Oncology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy;
| | - Niccolò Innocenti
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (V.N.); (N.I.)
| | - Veronica Saletti
- Developmental Neurology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy;
| | - Antonella Potenza
- Neurobiology Laboratory, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (A.P.); (T.C.); (G.P.); (G.G.); (L.G.)
| | - Tatiana Carrozzini
- Neurobiology Laboratory, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (A.P.); (T.C.); (G.P.); (G.G.); (L.G.)
| | - Giuliana Pollaci
- Neurobiology Laboratory, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (A.P.); (T.C.); (G.P.); (G.G.); (L.G.)
| | - Gemma Gorla
- Neurobiology Laboratory, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (A.P.); (T.C.); (G.P.); (G.G.); (L.G.)
| | - Rita Paroni
- Department of Health Sciences, Università degli Studi di Milano, 20142 Milan, Italy; (M.D.C.); (R.P.)
| | - Riccardo Ghidoni
- Neurorehabilitation Department, IRCCS Istituti Clinici Scientifici Maugeri, 20138 Milan, Italy;
| | - Laura Gatti
- Neurobiology Laboratory, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy; (A.P.); (T.C.); (G.P.); (G.G.); (L.G.)
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Muramatsu K, Tani Y, Seto T, Iwanaga R, Mihara A, Ihara K, Sakai T. Schwannoma in the extremity: clinical features and microscopic intra-capsular enucleation. J Rural Med 2021; 16:184-190. [PMID: 34707726 PMCID: PMC8527622 DOI: 10.2185/jrm.2021-020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2021] [Accepted: 05/31/2021] [Indexed: 11/27/2022] Open
Abstract
Objective: Schwannomas are the most common type of neoplasm of the
peripheral nerves. Enucleation is a standard surgical procedure; however, it occasionally
results in iatrogenic nerve injury, even with atraumatic procedures. Herein, we present
the clinical characteristics of schwannoma arising in the extremities and discuss the
clinical outcomes of extra- and intra-capsular
enucleation. Patients and Methods: We reviewed 122 schwannomas treated at our institute.
Schwannomas arising from the minor nerve (n=30) or intramuscularly (n=15) were operated
using the extra-capsular technique. Of the 77 major nerve schwannomas, 62 schwannomas were
treated using the intra-capsular technique and 15 schwannomas using the extra-capsular
technique. Results: Neurological deficits following enucleation were significantly
lower using the intra-capsular technique than with the extra-capsular technique. The
patient age, duration of symptoms, maximum tumor diameter, and site of occurrence were not
associated with subsequent neurological deficits. With both techniques, no tumor
recurrence was observed at the final follow-up. Conclusion: These results support the use of intra-capsular
micro-enucleation as a safe and reliable treatment for every type of schwannoma. To
minimize the risk of nerve injury, en bloc resection should not be used because the main
purpose of schwannoma surgery is the relief of symptoms, not tumor resection.
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Affiliation(s)
- Keiichi Muramatsu
- Department of Hand and Microsurgery, Nagato General Hospital, Japan.,Department of Orthopedic Surgery, Yamaguchi University School of Medicine, Japan
| | - Yasuhiro Tani
- Department of Hand and Microsurgery, Nagato General Hospital, Japan
| | - Tetsuya Seto
- Department of Hand and Microsurgery, Nagato General Hospital, Japan
| | - Ryuta Iwanaga
- Department of Orthopedic Surgery, Yamaguchi University School of Medicine, Japan
| | - Atsushi Mihara
- Department of Orthopedic Surgery, Yamaguchi University School of Medicine, Japan
| | - Koichiro Ihara
- Department of Orthopedic Surgery, Kanmon Medical Center, Japan
| | - Takashi Sakai
- Department of Orthopedic Surgery, Yamaguchi University School of Medicine, Japan
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Fader F, Mohamad Yunus MR, Mat Baki M. Recurrent laryngeal nerve schwannoma: same setting of resection and injection laryngoplasty. BMJ Case Rep 2021; 14:e245193. [PMID: 34706913 PMCID: PMC8552129 DOI: 10.1136/bcr-2021-245193] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/10/2021] [Indexed: 11/04/2022] Open
Abstract
A 33-year-old woman was diagnosed with right recurrent laryngeal nerve (RLN) schwannoma. She presented with a long history of hoarseness, and only recently developed dysphagia. On physical examination, a mass was observed over the right cervical level IV. Endoscopic examination of the larynx showed that she had right unilateral vocal cord palsy. She successfully underwent transcervical resection of the tumour followed by injection laryngoplasty. This study discusses the presentation of the tumour, radiological findings, our working diagnosis and treatment options of RLN schwannoma.
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Affiliation(s)
- Farhan Fader
- Department of Otorhinolaryngology, National University of Malaysia Faculty of Medicine, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia
| | - Mohd Razif Mohamad Yunus
- Department of Otorhinolaryngology, National University of Malaysia Faculty of Medicine, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia
| | - Marina Mat Baki
- Department of Otorhinolaryngology, National University of Malaysia Faculty of Medicine, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia
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48
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Sturdà C, Pennisi G, D'Alessandris QG, Mattogno P, Fernandez E, Granata G, Gessi M, Lauretti L. Multinodular plexiform tumors of major peripheral nerves: A practical overview. J Clin Neurosci 2021; 93:106-111. [PMID: 34656232 DOI: 10.1016/j.jocn.2021.09.022] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2021] [Revised: 09/06/2021] [Accepted: 09/09/2021] [Indexed: 11/26/2022]
Abstract
BACKGROUND AND AIMS Multinodular/plexiform schwannomas and neurofibromas of major nerves are rare: before surgery, differential diagnosis among these two uncommon variants is challenging. For both forms, surgical removal is recommended in case of progressive growth and worsening of neurological symptoms. Surgery has a higher risk of neurological damage than conventional schwannomas or neurofibromas. In literature, a comparison among these rare tumors is usually limited to the pathological aspect while specific surgical and clinical management indications are lacking. Cutaneous tumors of both forms arising from terminal peripheral nerves' branches might be treated by plastic surgeons while tumors of major nerves remain under neurosurgical competence. Here we report our recent neurosurgical experience on the matter, to furnish useful suggestions for the management of these tumors. METHOD We analyzed the clinical, radiological, and pathological data in a consecutive case series of plexiform/multinodular nerve tumors operated at our institution in the last five years. RESULTS In our series, neurofibroma type of plexiform tumors was more frequent than schwannoma type: two sporadic plexiform-multinodular schwannomas (patients 1, and 5) and three multinodular/plexiform Neurofibromatosis familial (Neurofibromatosis 1 / NF-1) (patients 2, 3, and 4). Surgery was complex when major nerves were involved. The early outcome appeared mostly related to the pre-surgical neurological conditions and histological grading. INTERPRETATION Although sharing some features, multinodular-plexiform schwannomas and neurofibromas have consistent differences from the clinical, surgical and pathological points of view.
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Affiliation(s)
- Cosimo Sturdà
- Department of Neuroscience, Section of Neurosurgery, Catholic University School of Medicine, Rome, Italy
| | - Giovanni Pennisi
- Department of Neuroscience, Section of Neurosurgery, Catholic University School of Medicine, Rome, Italy; Neurosurgery Unit, Fondazione Policlinico Universitario "Agostino Gemelli" - IRCCS, Rome, Italy.
| | | | - Pierpaolo Mattogno
- Neurosurgery Unit, Fondazione Policlinico Universitario "Agostino Gemelli" - IRCCS, Rome, Italy
| | - Eduardo Fernandez
- Neurosurgery Unit, Fondazione Policlinico Universitario "Agostino Gemelli" - IRCCS, Rome, Italy
| | - Giuseppe Granata
- Unit of Neurology, Fondazione Policlinico Universitario "Agostino Gemelli" - IRCCS, Rome, Italy
| | - Marco Gessi
- Unit of NeuroPathology, Fondazione Policlinico Universitario "Agostino Gemelli" - IRCCS, Rome, Italy
| | - Liverana Lauretti
- Department of Neuroscience, Section of Neurosurgery, Catholic University School of Medicine, Rome, Italy; Neurosurgery Unit, Fondazione Policlinico Universitario "Agostino Gemelli" - IRCCS, Rome, Italy
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49
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Wilson TJ, Hamrick F, Alzahrani S, Dibble CF, Koduri S, Pendleton C, Saleh S, Ali ZS, Mahan MA, Midha R, Ray WZ, Yang LJS, Zager EL, Spinner RJ. Analysis of the effect of intraoperative neuromonitoring during resection of benign nerve sheath tumors on gross-total resection and neurological complications. J Neurosurg 2021; 135:1231-1240. [PMID: 33578389 DOI: 10.3171/2020.8.jns202885] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2020] [Accepted: 08/26/2020] [Indexed: 11/06/2022]
Abstract
OBJECTIVE The aim of this study was to examine the role of intraoperative neuromonitoring (IONM) during resection of benign peripheral nerve sheath tumors in achieving gross-total resection (GTR) and in reducing postoperative neurological complications. METHODS Data from consecutive adult patients who underwent resection of a benign peripheral nerve sheath tumor at 7 participating institutions were combined. Propensity score matching was used to balance covariates. The primary outcomes of interest were the association between IONM and GTR and the association of IONM and the development of a permanent postoperative neurological complication. The secondary outcomes of interest were the association between IONM and GTR and the association between IONM and the development of a permanent postoperative neurological complication in the subgroup of patients with tumors involving a motor or mixed nerve. Univariate and multivariate logistic regression were then performed on the propensity score-matched samples to assess the ability of the independent variables to predict the outcomes of interest. RESULTS A total of 337 patients who underwent resection of benign nerve sheath tumors were included. In multivariate analysis, the use of IONM (OR 0.460, 95% CI 0.199-0.978; p = 0.047) was a significant negative predictor of GTR, whereas none of the variables, including IONM, were associated with the occurrence of a permanent postoperative neurological complication. Within the subgroup of motor/mixed nerve tumors, in the multivariate analysis, IONM (OR 0.263, 95% CI 0.096-0.723; p = 0.010) was a significant negative predictor of a GTR, whereas IONM (OR 3.800, 95% CI 1.925-7.502; p < 0.001) was a significant positive predictor of a permanent postoperative motor deficit. CONCLUSIONS Overall, 12% of the cohort had a permanent neurological complication, with new or worsened paresthesias most common, followed by pain and then weakness. The authors found that formal IONM was associated with a reduced likelihood of GTR and had no association with neurological complications. The authors believe that these data argue against IONM being considered standard of care but do not believe that these data should be used to universally argue against IONM during resection of benign nerve sheath tumors.
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Affiliation(s)
- Thomas J Wilson
- 1Department of Neurosurgery, Stanford University, Stanford, California
| | - Forrest Hamrick
- 2Department of Neurosurgery, University of Utah, Salt Lake City, Utah
| | - Saud Alzahrani
- 3Department of Clinical Neurosciences, University of Calgary, Alberta, Canada
| | | | - Sravanthi Koduri
- 5Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan
| | | | - Sara Saleh
- 5Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan
| | - Zarina S Ali
- 7Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Mark A Mahan
- 2Department of Neurosurgery, University of Utah, Salt Lake City, Utah
| | - Rajiv Midha
- 3Department of Clinical Neurosciences, University of Calgary, Alberta, Canada
| | - Wilson Z Ray
- 4Department of Neurosurgery, Washington University in St. Louis, Missouri
| | - Lynda J S Yang
- 5Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan
| | - Eric L Zager
- 7Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Robert J Spinner
- 6Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota; and
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50
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Pressney I, Khoo M, Khan R, Abernethy P, Hargunani R, Saifuddin A. Morphology of the entering and exiting nerve as a differentiating feature of benign from malignant peripheral nerve sheath tumours of the brachial plexus. Skeletal Radiol 2021; 50:1557-1565. [PMID: 33410965 DOI: 10.1007/s00256-020-03689-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/31/2020] [Revised: 11/28/2020] [Accepted: 11/29/2020] [Indexed: 02/02/2023]
Abstract
OBJECTIVE To identify if morphology of the entering and exiting nerve involved by a nerve sheath tumour in the brachial plexus can help differentiate between benign (B) and malignant (M) peripheral nerve sheath tumours (PNSTs). MATERIALS AND METHODS Retrospective review of 85 patients with histologically confirmed primary PNSTs of the brachial plexus over a 12.5-year period. Clinical data and all available MRI studies were independently evaluated by 2 consultant musculoskeletal radiologists blinded to the final histopathological diagnosis assessing for maximal lesion dimension, visibility and morphology of the entering and exiting nerve, and other well-documented features of PNSTs. RESULTS The study included 47 males and 38 females with mean age 46.7 years (range, 8-81 years). There were 73 BPNSTs and 12 MPNSTs. The entering nerve was not identified in 5 (7%), was normal in 17 (23%), was tapered in 38 (52%) and showed lobular enlargement in 13 (18%) BPNSTs compared with 0 (0%), 0 (0%), 2 (17%) and 10 (83%) MPNSTs respectively. The exiting nerve was not identified in 5 (7%), was normal in 20 (27%), was tapered in 42 (58%) and showed lobular enlargement in 6 (8%) BPNSTs compared with 4 (33%), 0 (0%), 2 (17%) and 6 (50%) MPNSTs respectively. Increasing tumour size, entering and exiting nerve morphology and suspected MRI diagnosis were statistically significant differentiators between BPNST and MPNST (p < 0.001). IOC for nerve status was poor to fair but improved to good if normal/tapered appearance were considered together with improved specificity of 81-91% for BPNST and sensitivity of 75-83%. CONCLUSIONS Morphology of the adjacent nerve is a useful additional MRI feature for distinguishing BPNST from MPNST of the brachial plexus.
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Affiliation(s)
- I Pressney
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK.
| | - M Khoo
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK
| | - R Khan
- Department of Radiology, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates
| | - P Abernethy
- Department of Radiology, University Hospitals Plymouth NHS Trust, Plymouth, UK
| | - R Hargunani
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK
| | - A Saifuddin
- Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK
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