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Lam CSA, Martins Coelho VDP, Wilson S, Palmer J, Bardeesi A, Chakravarthy V. Hybrid therapy and use of carbon-fiber-reinforced polyetheretherketone instrumentation for management of mobile spine chordomas: A case series and review of the literature. Surg Neurol Int 2025; 16:130. [PMID: 40353162 PMCID: PMC12065495 DOI: 10.25259/sni_53_2025] [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: 01/18/2025] [Accepted: 03/06/2025] [Indexed: 05/14/2025] Open
Abstract
Background Mobile spine chordomas demonstrate varied surgical risk profiles compared to their sacral analogs. Often, the limitation to performing an en bloc resection of a mobile spine chordoma is tumor violation of the epidural space. Given these limitations, we propose the utilization of carbon fiber-reinforced polyetheretherketone (CFR-PEEK) instrumentation in separation surgery to enhance visualization for stereotactic body radiation therapy (SBRT) planning, allowing an ablative radiosurgical dose to be delivered. Methods We present two illustrative cases highlighting the advantages of hybrid therapy (separation surgery and adjuvant SBRT) with CFR-PEEK instrumentation in the management of mobile spine chordoma. Results Case 1 is a 62-year-old female with an L4 chordoma who underwent separation surgery and L3-5 posterior instrumented fusion using CFR-PEEK instrumentation. Case 2 is a 68-year-old female with a L3 chordoma who underwent revision separation surgery encompassing completion of L3 partial corpectomy and CFR-PEEK screw exchange of prior L2-4 titanium instrumentation. Both patients received postoperative ablative SBRT. At 18-month postoperative time points, both patients were clinically stable, with no signs of tumor recurrence or progression. Conclusion Mobile spine chordomas present a unique challenge in obtaining a margin negative en bloc resection. Separation surgery allows the ability to decrease surgical morbidity and deliver an ablative radiosurgical dose. Furthermore, the incorporation of CFR-PEEK instrumentation allows the utilization of multiparametric magnetic resonance imaging for long-term disease monitoring. Hybrid therapy, a less morbid alternative to standard en bloc spondylectomy, offers a better surgical morbidity profile by combining effectively with SBRT for optimal tumor control.
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Affiliation(s)
- Chi Shing Adrian Lam
- Department of Neurological Surgery, The Ohio State University Wexner Medical Center, United States
| | | | - Seth Wilson
- Department of Neurological Surgery, The Ohio State University Wexner Medical Center, United States
| | - Joshua Palmer
- Department of Radiation Oncology, The Ohio State University Comprehensive Cancer Center, Columbus, Ohio, United States
| | - Anas Bardeesi
- Department of Neurological Surgery, The Ohio State University Wexner Medical Center, United States
| | - Vikram Chakravarthy
- Department of Neurological Surgery, The Ohio State University Wexner Medical Center, United States
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Bosman EM, Keizer ME, van Aalst J, Broen MP, Postma AA, Vernemmen AI, Kunst HP, Temel Y. Spontaneous Shrinking and Growing Skull Base Chordoma. J Neurol Surg Rep 2025; 86:e107-e111. [PMID: 40352577 PMCID: PMC12064314 DOI: 10.1055/a-2587-6573] [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: 02/18/2025] [Accepted: 04/09/2025] [Indexed: 05/14/2025] Open
Abstract
Background Chordomas are rare slow-growing tumors occurring in the axial skeleton and can demonstrate local aggressive behavior, typically extending from the median axis, compressing surrounding tissue. Complete surgical resection and adjuvant radiotherapy are the preferred treatments. We present an unusual case of a spontaneously shrinking and growing off-midline petroclival chordoma. Case Description A 23-year-old woman presented with right abducens nerve palsy. Computed tomography and magnetic resonance imaging (MRI) revealed an off-midline petroclival lesion compressing the abducens nerve with characteristics of a chondrosarcoma. Preoperative MRI indicated spontaneous lesion regression, and the abducens nerve showed clinical improvement. Hence, the planned surgery was canceled. During the wait-and-scan period, abducens nerve palsy recurred. MRI confirmed lesion growth and showed an intratumoral linear structure indicative of blood. Even though preoperative MRI again demonstrated shrinkage, the lesion was surgically removed. Despite the unusual presentation, histopathological examination diagnosed a conventional chordoma. A second surgery was required to remove the residual tumor, after which the patient received high-dose photon beam therapy. Conclusion This article discusses the uncommon presentation and behavior of a petroclival chordoma, showing fluctuating cycles of off-midline growth and spontaneous regression. While intratumoral hemorrhage is hypothesized to explain this tumor behavior, the exact etiology needs further investigation. The case presented here emphasizes the importance of considering chordoma in the differential diagnosis despite an atypical disease course.
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Affiliation(s)
- Esmée M. Bosman
- Department of Neurosurgery, Maastricht University Medical Center+, Maastricht, The Netherlands
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
| | - Max E. Keizer
- Department of Neurosurgery, Maastricht University Medical Center+, Maastricht, The Netherlands
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
- School for Mental Health and Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, The Netherlands
| | - Jasper van Aalst
- Department of Neurosurgery, Maastricht University Medical Center+, Maastricht, The Netherlands
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
| | - Martinus P.G. Broen
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
- Department of Neurology, Maastricht University Medical Center+, Maastricht, The Netherlands
| | - Alida A. Postma
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
- School for Mental Health and Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, The Netherlands
- Department of Radiology, Maastricht University Medical Center+, Maastricht, The Netherlands
| | - Astrid I.P. Vernemmen
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
- Department of Pathology, Maastricht University Medical Center, Maastricht, The Netherlands
| | - Henricus P.M. Kunst
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
- School for Mental Health and Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, The Netherlands
- Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands
- Department of Otorhinolaryngology, Maastricht University Medical Center+, Maastricht, The Netherlands
| | - Yasin Temel
- Department of Neurosurgery, Maastricht University Medical Center+, Maastricht, The Netherlands
- Dutch Academic Alliance Skull Base Pathology, Maastricht University Medical Center+ and Radboud University Medical Center, Maastricht and Nijmegen, The Netherlands
- School for Mental Health and Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, The Netherlands
- Istanbul Atlas University, Faculty of Medicine, Istanbul, Türkiye
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Peels J, Vulsteke C, Boedts M, Marin E, De Cocker LJL. Delayed FLAIR-enhancement of benign notochordal remnant (ecchordosis physaliphora). Neuroradiol J 2025; 38:220-223. [PMID: 39601196 PMCID: PMC11600419 DOI: 10.1177/19714009241303131] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/18/2024] [Revised: 11/05/2024] [Accepted: 11/12/2024] [Indexed: 11/29/2024] Open
Abstract
Ecchordosis physaliphora (EP) is a benign notochordal remnant most commonly encountered in the skull base. In opposition to typical cases of its invasive counterpart, that is, chordoma, EP does not show T1-enhancement. Now, we describe three patients with EP, discovered on delayed contrast-enhanced 3D FLAIR performed for endolymphatic hydrops imaging in suspected Menière's disease. All EP cases demonstrated intense, delayed FLAIR-enhancement of the notochordal remnant. The importance of this new observation of FLAIR-enhancement in EP is threefold: (1) it may increase the detection rate of EP, (2) FLAIR-enhancement in EP should not be mistaken for T1-enhancement in chordoma, and (3) it may open a new window for future imaging studies aiming to better differentiate EP from chordoma.
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Affiliation(s)
- Jeroen Peels
- Department of Radiology, AZ Maria Middelares, Ghent, Belgium
| | - Christof Vulsteke
- Department of Medical Oncology, Integrated Cancer Center Ghent, AZ Maria Middelares, Belgium
- Center for Oncological Research (CORE), Integrated Personalized and Precision Oncology Network (IPPON), University of Antwerp, Belgium
| | - Michael Boedts
- Ear, Nose and Throat Department, AZ Maria Middelares, Ghent, Belgium
| | - Eline Marin
- Ear, Nose and Throat Department, AZ Maria Middelares, Ghent, Belgium
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Charan BD, Agarwal S, Singh E, Jain S, Das S, Garg A, Sebastian LJD, Singh M. Nonenhancing Prepontine Chordoma with Diffusion Restriction Mimicking an Epidermoid Cyst. Asian J Neurosurg 2025; 20:138-142. [PMID: 40041598 PMCID: PMC11875700 DOI: 10.1055/s-0044-1791581] [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] [Indexed: 03/06/2025] Open
Abstract
Intradural prepontine chordoma is an extremely rare entity and depicts benign features with no recurrence and has a good prognosis as compared with bone endogenous chordoma. Radiologically, it may mimic an epidermoid cyst and present an imaging challenge. Here we present a case of a middle adolescent girl who presented with headache along with limb and facial weakness. A nonenhancing, diffusion-restricting, predominantly intradural cystic-appearing mass lesion was seen in the prepontine cistern. Initially, an epidermoid cyst was suspected, but histopathology revealed it to be a chordoma. Destruction of the clivus is important and chordoma should be included in differential diagnosis.
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Affiliation(s)
- Bheru Dan Charan
- Neuroimaging and Interventional Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
- Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
| | - Sushant Agarwal
- Guwahati Medical College and Hospital, Guwahati, Assam, India
| | - Ekamjot Singh
- Neuroimaging and Interventional Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
| | - Savyasachi Jain
- Neuroimaging and Interventional Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
| | - Sumanta Das
- Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India
| | - Ajay Garg
- Neuroimaging and Interventional Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
| | | | - Manmohan Singh
- Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
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Al-Quwayee MK, Albadr F, Almasoud RN, Alghamdi N, Alhathloul ZA, Alquhidan M. Isolated Suprasellar Chordoma Mimicking Pituitary Adenoma: A Case Report. Cureus 2025; 17:e80647. [PMID: 40236339 PMCID: PMC11998626 DOI: 10.7759/cureus.80647] [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: 03/16/2025] [Indexed: 04/17/2025] Open
Abstract
Chordomas are uncommon neoplasms predominantly observed in adult males, typically middle-aged. These tumors present with diverse clinical features, including headaches, cranial neuropathies, and cerebrospinal fluid leakage. Surgical intervention is often complex due to tumor location or postresection reconstruction requirements, necessitating a multidisciplinary approach for optimal management. The report describes an atypical case of a 20-year-old female patient presenting with persistent headaches and visual disturbances. Magnetic resonance imaging (MRI) revealed a large sellar/suprasellar mass initially suggestive of a pituitary adenoma. Following surgical excision, histopathological analysis confirmed a chordoma. This report emphasizes the diagnostic and therapeutic challenges of suprasellar chordomas, which may radiologically mimic common sellar lesions. The case underscores the importance of including chordoma in the differential diagnosis of sellar/suprasellar masses, especially in younger patients with visual impairment and headache.
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Affiliation(s)
| | - Fahad Albadr
- Radiology and Medical Imaging, King Saud University Medical City, Riyadh, SAU
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6
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Sakai M, Hiyama T, Kuno H, Kobayashi T, Nakajima T. Imaging of the skull base and orbital tumors. Jpn J Radiol 2025; 43:152-163. [PMID: 39302526 PMCID: PMC11790709 DOI: 10.1007/s11604-024-01662-9] [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: 06/22/2024] [Accepted: 09/10/2024] [Indexed: 09/22/2024]
Abstract
The skull base and orbit have complicated anatomical structures where various tumors can occur. The tumor may present with neurological symptoms; however, its diagnosis is clinically difficult owing to accessibility issues. Therefore, diagnostic imaging is crucial in assessing tumors in the skull base and orbit and guiding subsequent management. Notably, some tumors have a predilection for a specific site of origin, and identifying the site of origin on imaging can help narrow the differential diagnosis. At the skull base, chordomas typically occur in the clivus, chondrosarcomas in the paramedian areas, paragangliomas in the jugular foramen, neurogenic tumors, and perineural spread in the neural foramen. Among orbital tumors, cavernous hemangiomas usually occur in the intraconal space, and pleomorphic adenomas and adenoid cystic carcinomas occur in the lacrimal glands. Some skull base and orbital tumors exhibit distinctive imaging features. Chordomas and chondrosarcomas of the skull base show high signal intensities on T2-weighted images, with chondrosarcomas often displaying cartilaginous calcifications. Paragangliomas are characterized by their hypervascular nature. In the orbit, cavernous hemangiomas and pleomorphic adenomas present unique dynamic patterns. Immunoglobulin G4-related disease forms lesions along the nerves. Identifying the tumor origin and its imaging characteristics can help narrow the differential diagnosis of skull base and orbital tumors.
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Affiliation(s)
- Masafumi Sakai
- Department of Radiology, Institute of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Takashi Hiyama
- Department of Diagnostic Radiology, National Cancer Center Hospital East, 6-5-1 Kashiwanoha, Kashiwa, Chiba, 277-8577, Japan.
| | - Hirofumi Kuno
- Department of Diagnostic Radiology, National Cancer Center Hospital East, 6-5-1 Kashiwanoha, Kashiwa, Chiba, 277-8577, Japan
| | - Tatsushi Kobayashi
- Department of Diagnostic Radiology, National Cancer Center Hospital East, 6-5-1 Kashiwanoha, Kashiwa, Chiba, 277-8577, Japan
| | - Takahito Nakajima
- Department of Radiology, Institute of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
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Miladinovic V, Krol ADG, Bloem JL, Bovée JVMG, Lam SW, Peul WC, Cañete AN, Verbist BM. Combining morphological and functional imaging parameters to diagnose primary bone neoplasms in the skull base, spine and sacrum. Skeletal Radiol 2025; 54:287-302. [PMID: 38967687 PMCID: PMC11652577 DOI: 10.1007/s00256-024-04742-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2024] [Revised: 06/24/2024] [Accepted: 06/27/2024] [Indexed: 07/06/2024]
Abstract
PURPOSE Morphological magnetic resonance (MR) and computed tomography (CT) features are used in combination with histology for diagnosis and treatment selection of primary bone neoplasms. Isolated functional MRI parameters have shown potential in diagnosis. Our goal is to facilitate diagnosis of primary bone neoplasms of the skull base, mobile spine and sacrum, by a comprehensive approach, combining morphological and functional imaging parameters. MATERIALS AND METHODS Pre-treatment MR of 80 patients with histologically proven diagnosis of a primary bone neoplasm of the skull base, mobile spine and sacrum were retrospectively analyzed for morphological and functional MRI parameters. Functional parameters were measured in 4 circular regions of interest per tumor placed on non-adjacent scan slices. Differences in values of functional parameters between different histologies were analyzed with Dunn's test. RESULTS Chordomas were the predominant histology (60.0%). Most neoplasms (80.0%) originated in the midline and had geographical (78.2%) bone destruction. Amorphous-type calcification (pre-existing bone) was seen only in chordomas. Homogeneous contrast enhancement pattern was seen only in chondrosarcoma and plasmacytoma. Ktrans and Kep were significantly lower in both chordoma, and chondrosarcoma compared to giant cell tumor of the bone (p = 0.006 - 0.011), and plasmacytoma (p = 0.004 - 0.014). Highest diffusion-weighted MRI apparent diffusion coefficient (ADC) values corresponded to chondrosarcoma and were significantly higher to those of chordoma (p = 0.008). CONCLUSION We identified the most discriminating morphological parameters and added functional MR parameters based on histopathological features that are useful in making a confident diagnosis of primary bone neoplasms in the skull base, mobile spine and sacrum.
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Affiliation(s)
- Vesna Miladinovic
- Department of Radiation Oncology, Leiden University Medical Center, Leiden, The Netherlands.
- HollandPTC, Delft, The Netherlands.
| | - Augustinus D G Krol
- Department of Radiation Oncology, Leiden University Medical Center, Leiden, The Netherlands
- HollandPTC, Delft, The Netherlands
| | - Johan L Bloem
- Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands
| | - Judith V M G Bovée
- Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
| | - Suk Wai Lam
- Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
| | - Wilco C Peul
- University Neurosurgical Center Holland, Leiden University Medical Center, Leiden, Zuid-Holland, Netherlands
| | - Ana Navas Cañete
- Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands
| | - Berit M Verbist
- Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands
- HollandPTC, Delft, The Netherlands
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8
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Geremew TT, Engida GG, Mihiret AK, Gelaw AD, Tesgera TG. A rare case of primary chordoma of the nasopharynx mimicking nasopharyngeal carcinoma: A case report. Int J Surg Case Rep 2025; 126:110779. [PMID: 39731799 PMCID: PMC11741022 DOI: 10.1016/j.ijscr.2024.110779] [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: 11/11/2024] [Revised: 12/12/2024] [Accepted: 12/22/2024] [Indexed: 12/30/2024] Open
Abstract
INTRODUCTION AND IMPORTANCE Chordoma is an uncommon malignant tumor that originates from the remnants of the primitive notochord in the embryo. They account for 1 % of intracranial tumors and 4 % of primary bone tumors. It is a locally aggressive tumor with a low risk of metastasis. It is more common in males than in females and is more common in the age range of 40-60. It most commonly occurs in the sacrococcygeal areas but can occur in uncommon locations, such as the dental area and nasopharynx. This case is important because there are few case reports in the world. CASE PRESENTATION Here we present a 60 years old male patient presented with foul smelling nasal discharge, nasal obstruction and snoring of 2 years duration and Head CT scan was concluded to be advanced nasopharyngeal carcinoma but histopathology result turned out to be the uncommon nasopharyngeal chordoma. Therefore, he was treated with surgical excision and adjuvant radiotherapy. CLINICAL DISCUSSION Patient presentation usually varies by location; however, patients can be asymptomatic. CT and MRI are considered the initial modes of investigation, but histopathology is needed to confirm the diagnosis. Surgery is the primary mode of treatment with or without adjuvant chemoradiotherapy. CONCLUSION Even though the typical location of chordoma is the sacrococcygeal area of the body, it can arise in uncommon locations, such as the nasopharynx.
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Affiliation(s)
- Teketel Tadesse Geremew
- Department of Pathology, Hawassa University Comprehensive Specialized Hospital, Hawassa, Sidama, Ethiopia.
| | - Ghion Getenet Engida
- Department of Pathology, Hawassa University Comprehensive Specialized Hospital, Hawassa, Sidama, Ethiopia
| | - Adugnaw Kindu Mihiret
- Department of Internal Medicine, University of Gondar, College of Medicine and Health Science, Ethiopia
| | - Abeje Diress Gelaw
- Department of Radiology, University of Gondar, College of Medicine and Health Science, Ethiopia
| | - Tigist Gutema Tesgera
- Department of Internal medicine, Hawassa University Comprehensive Specialized Hospital, Hawassa, Sidama, Ethiopia
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9
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Jaumally BA, Balachandran DD, Salem AM, Bashoura L, Faiz SA. Central sleep apnea (CSA) in a treated brainstem chordoma. Sleep Med X 2024; 8:100127. [PMID: 39974506 PMCID: PMC11838080 DOI: 10.1016/j.sleepx.2024.100127] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2022] [Revised: 09/21/2024] [Accepted: 10/02/2024] [Indexed: 02/21/2025] Open
Affiliation(s)
- Bibi Aneesah Jaumally
- Division of Pulmonary, Allergy and Critical Care Medicine, The University of Alabama at Birmingham, Alabama, USA
| | - Diwakar D. Balachandran
- Department of Pulmonary Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Ahmed M. Salem
- Division of Pulmonary, Allergy and Critical Care Medicine, The University of Alabama at Birmingham, Alabama, USA
| | - Lara Bashoura
- Department of Pulmonary Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Saadia A. Faiz
- Department of Pulmonary Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
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10
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Lee S, Teferi N, Vivanco-Suarez J, Chowdhury A, Glennon S, Kato K, Matern T, Eschbacher KL, Petronek M, Hitchon P. Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature. NORTH AMERICAN SPINE SOCIETY JOURNAL 2024; 20:100569. [PMID: 39687058 PMCID: PMC11648787 DOI: 10.1016/j.xnsj.2024.100569] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/04/2024] [Revised: 10/13/2024] [Accepted: 10/26/2024] [Indexed: 12/18/2024]
Abstract
Background Chordomas are rare, slow growing, locally aggressive malignant bone tumors that arise from remnants of the embryonic notochord with variable presenting symptoms depending on tumor location. Methods All patients with craniospinal chordoma managed at our institution between 1982 and 2023 were retrospectively reviewed. Demographics, tumor characteristics, clinical course and treatment, and long-term neurological and survival outcomes were collected. Adjuvant radiotherapy (RT) was stratified into standard dose fractionated radiotherapy (standard XRT) for doses of 50 to 60 Gy at 1.8 Gy fractions or high dose hyperfractionated stereotactic radiotherapy (HD-FSRT) for doses of 60 to 81 Gy at 1.2-1.5 Gy fractions per treatment. Descriptive statistics, univariate analysis, Log-rank test, and Kaplan-Meier survival analysis were performed. Results A total of 37 patients were included in our cohort (mean age 46.0 ± 20.8 years; 22 male). Clival chordomas accounted for the majority of patients (56.8%), followed by vertebral (27%) and sacral (10.8%) chordomas. Thirty-five patients (94.6%) underwent gross total resection (GTR) or subtotal resection (STR), and 2 patients underwent excisional biopsy only. Postoperatively, functional status trended towards improvement (KPS: Preop- 80 [range 40-100] vs. Post op- 90 [60-100], p = .0911) and all patients either maintained or improved their neurological function. Median overall survival (OS) after diagnosis was 16.5 years. Age < 65, clival tumor location, post-operative Frankel grade E, and administration of adjuvant RT following initial STR significantly improved OS. OS of GTR patients was not significantly affected by adjuvant RT treatment. Conclusions Our results show the best long-term survival outcomes for chordoma patients undergoing GTR of tumor tissue. Higher postoperative neurological function was significantly associated with OS, highlighting the importance of maximal but safe total tumor resection. Moreover, adjuvant RT improved long-term survival for patients that underwent STR but had no effect on survival outcomes for patients that underwent GTR.
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Affiliation(s)
- Sarah Lee
- Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Nahom Teferi
- Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Juan Vivanco-Suarez
- Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Ajmain Chowdhury
- University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Stephen Glennon
- Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Kyle Kato
- University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Tyson Matern
- Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Kathryn L. Eschbacher
- Department of Pathology, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Michael Petronek
- Department of Radiation Oncology, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
| | - Patrick Hitchon
- Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States
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11
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Potter GM, Siripurapu R, Herwadkar A, Abdulla S, Ikotun O, Broadhurst P, Woodward M, Bhalla RK, Glancz LJ, Hammerbeck-Ward CL, Rutherford SA, Pathmanaban ON, Roncaroli F, Colaco RJ, Pan S, Whitfield GA. Skull Base Chordoma and Chondrosarcoma: Neuroradiologist's Guide to Diagnosis, Surgical Management, and Proton Beam Therapy. Radiographics 2024; 44:e240036. [PMID: 39298353 DOI: 10.1148/rg.240036] [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: 09/21/2024]
Abstract
Skull base chordomas and chondrosarcomas are distinct types of rare, locally aggressive mesenchymal tumors that share key principles of imaging investigation and multidisciplinary care. Maximal safe surgical resection is the treatment choice for each, often via an expanded endoscopic endonasal approach, with or without multilayer skull base repair. Postoperative adjuvant radiation therapy is frequently administered, usually with particle therapy such as proton beam therapy (PBT). Compared with photon therapy, PBT enables dose escalation while limiting damage to dose-limiting neurologic structures, particularly the brainstem and optic apparatus, due to energy deposition being delivered at a high maximum with a rapid decrease at the end of the penetration range (Bragg peak phenomenon). Essential requirements for PBT following gross total or maximal safe resection are tissue diagnosis, minimal residual tumor after resection, and adequate clearance from PBT dose-limiting structures. The radiologist should understand surgical approaches and surgical techniques, including multilayer skull base repair, and be aware of evolution of postsurgical imaging appearances over time. Accurate radiologic review of all relevant preoperative imaging examinations and of intraoperative and postoperative MRI examinations plays a key role in management. The radiology report should reflect what the skull base surgeon and radiation oncologist need to know, including distance between the tumor and PBT dose-limiting structures, tumor sites that may be difficult to access via the endoscopic endonasal route, the relationship between intradural tumor and neurovascular structures, and tumor sites with implications for postresection stability. ©RSNA, 2024 Supplemental material is available for this article.
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Affiliation(s)
- Gillian M Potter
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Rekha Siripurapu
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Amit Herwadkar
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Sarah Abdulla
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Oluwaseun Ikotun
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Philip Broadhurst
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Mark Woodward
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Rajiv K Bhalla
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Laurence J Glancz
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Charlotte L Hammerbeck-Ward
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Scott A Rutherford
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Omar N Pathmanaban
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Federico Roncaroli
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Rovel J Colaco
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Shermaine Pan
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
| | - Gillian A Whitfield
- From the Department of Radiology (G.M.P., P.B., M.W.) and ENT Surgery (R.K.B.), Manchester Royal Infirmary, Oxford Road, Manchester, United Kingdom M13 9WL; Department of Neuroradiology, Walton Centre NHS Foundation Trust, Liverpool, United Kingdom (R.S.); Department of Radiology, Salford Royal Hospital, Salford, United Kingdom (A.H., S.A.); Department of Neurosurgery, Salford Royal Hospital, Salford, United Kingdom (S.A.R., O.N.P.); University Hospital Lewisham, London, United Kingdom (O.I.); Department of Neurosurgery, Queen's Medical Centre, Nottinghamshire, United Kingdom and University of Nottingham, Nottingham, United Kingdom (L.J.G.); Department of Neurosurgery, Royal Sussex County Hospital Brighton, United Kingdom (C.L.H-W.); Division of Neuroscience (F.R.) and Division of Cancer Sciences (G.A.W.), University of Manchester, Manchester, United Kingdom; and Department of Clinical Oncology, Christie Hospital, Manchester, United Kingdom (R.J.C., S.P., G.A.W.)
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Alsubaie H, Aldoseri R, Alshehabi M, Nasser M. Nasopharyngeal Chordoma: A Case Report. Cureus 2024; 16:e58636. [PMID: 38644942 PMCID: PMC11032088 DOI: 10.7759/cureus.58636] [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: 04/20/2024] [Indexed: 04/23/2024] Open
Abstract
Chordoma is a rare malignant neoplasm arising from remnants of primitive notochord. The most common location for chordoma is in the sacrum. This case presents a 10-year-old medically free male who came to the ENT clinic with the impression of adenoid hypertrophy. After further investigations, including imaging and biopsy, it was found to be a nasopharyngeal chordoma. Our aim, in this case, is to increase the suspension of differential diagnosis of nasopharyngeal masses other than adenoid hypertrophy. In addition, it highlights the importance of imaging in the evaluation of nasopharyngeal masses.
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Affiliation(s)
| | | | | | - Mai Nasser
- Otolaryngology & Head and Neck Surgery, The Royal Medical Services, Riffa, BHR
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13
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Pellerano FM, Messilaty MS, Chin SS, Parnes GJ, Akbar NA, Agarwal V, Mbekeani JN. Isolated Third Nerve Palsy as the Initial Manifestation of a Large Clival Chordoma. J Neuroophthalmol 2024; 44:e117-e119. [PMID: 36562553 DOI: 10.1097/wno.0000000000001764] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Affiliation(s)
- Fernando M Pellerano
- Department of Ophthalmology (FMP), Indiana University School of Medicine, Indianapolis, Indiana; Departments of Surgery (Ophthalmology) (MSM, JNM), and Radiology (GJP), Jacobi Medical Center, Bronx, New York; Department of Pathology (SSC), Montefiore Medical Center, Bronx, New York; Departments of Otorhinolaryngology (NAA, VA) and Neurosurgery (VA), Montefiore Medical Center, Bronx, New York; and Department of Ophthalmology and Visual Sciences (JNM), Albert Einstein College of Medicine, Bronx, New York
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14
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Tison T, Cespivova M, Brichard B, Huart C, Dumitriu D, Di Perri D. A rare case of pediatric extraosseous chordoma of the nasopharynx. Pediatr Blood Cancer 2024; 71:e30776. [PMID: 37985694 DOI: 10.1002/pbc.30776] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/27/2023] [Accepted: 10/30/2023] [Indexed: 11/22/2023]
Affiliation(s)
- Thaïs Tison
- Department of Radiation Oncology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Marketa Cespivova
- Department of Anatomopathology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Bénédicte Brichard
- Department of Pediatric Oncology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Caroline Huart
- Department of Othorhinolaryngology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Dana Dumitriu
- Department of Radiology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Dario Di Perri
- Department of Radiation Oncology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
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15
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Li B, Kim MG, Dominguez JF, Feldstein E, Kleinman G, Al-Mufti F, Kim M, Hanft S. Intrasellar hemorrhagic chordoma masquerading as pituitary apoplexy: case report and review of the literature. Br J Neurosurg 2023; 37:1685-1688. [PMID: 34148480 DOI: 10.1080/02688697.2021.1941761] [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: 04/01/2021] [Accepted: 06/07/2021] [Indexed: 10/21/2022]
Abstract
BACKGROUND AND IMPORTANCE Chordomas are centrally located, expansile soft tissue neoplasms that arise from the remnants of the embryological notochord. Hemorrhagic presentation is exceedingly rare and can resemble pituitary apoplexy. Moreover, a purely intrasellar location of a chordoma is extremely uncommon. We report a case of a hemorrhagic intrasellar chordoma in an adult male, which presented similarly to pituitary apoplexy and was resolved with surgical resection. CLINICAL PRESENTATION A 69-year-old male presented with a 4 week history of acute onset headache and concurrent diplopia, with significantly reduced testosterone and slightly reduced cortisol. His left eye demonstrated a sixth cranial nerve palsy. Magnetic resonance imaging of the brain showed a large hemorrhagic mass in the pituitary region with significant compression of the left cavernous sinus and superior displacement of the pituitary gland. The patient underwent an endoscopic endonasal transsphenoidal approach for the resection of the lesion. Near total resection was achieved. Final pathology revealed chordoma with evidence of intratumoral hemorrhage, further confirmed by immunopositive stain for brachyury. Post-operatively, the patient had improved diplopia and was discharged home on low dose hydrocortisone. At 3-month follow-up, his diplopia was resolved and new MRI showed stable small residual disease. CONCLUSIONS Apoplectic chordomas are uncommon given chordoma's characteristic lack of intralesional vascularity and represent a diagnostic challenge in the sellar region. Our unique case demonstrates that despite our initial impression of pituitary apoplexy, this was ultimately a case of apoplectic chordoma that responded well to endoscopic endonasal surgery.
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Affiliation(s)
- Boyi Li
- New York Medical College School of Medicine, Valhalla, NY, USA
| | - Michael G Kim
- Department of Neurosurgery, Westchester Medical Center, New York Medical College, Valhalla, NY, USA
| | - Jose F Dominguez
- Department of Neurosurgery, Westchester Medical Center, New York Medical College, Valhalla, NY, USA
| | - Eric Feldstein
- New York Medical College School of Medicine, Valhalla, NY, USA
| | - George Kleinman
- Department of Pathology, Westchester Medical Center, New York Medical College, Valhalla, NY, USA
| | - Fawaz Al-Mufti
- Department of Neurosurgery, Westchester Medical Center, New York Medical College, Valhalla, NY, USA
| | - Matthew Kim
- Department of Otolaryngology, Westchester Medical Center, New York Medical College, Valhalla, NY, USA
| | - Simon Hanft
- Department of Neurosurgery, Westchester Medical Center, New York Medical College, Valhalla, NY, USA
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16
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Nkie VE, Martin S. Fibrous Dysplasia of the Clivus: Case Report and Literature Review. Cureus 2023; 15:e45417. [PMID: 37854736 PMCID: PMC10581508 DOI: 10.7759/cureus.45417] [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: 08/31/2023] [Accepted: 09/13/2023] [Indexed: 10/20/2023] Open
Abstract
Fibrous dysplasia is a benign, developmental bone disorder that causes fibrous replacement of normal skeletal tissue. This may lead to weakness, distortion, and tissue expansion. Fibrous dysplasia can occur anywhere in the body, including the craniofacial area. The clivus is a central skull bone formed by the bases of the sphenoid and occiput, respectively. The clivus is a rare, usually unrecognized, and seldom reported location for the development of fibrous dysplasia. Although fibrous dysplasia of the clivus (FDC) is usually discovered by incidental findings, it can sometimes present with clinical symptoms. In this case, we discuss a 30-year-old male who presents to the emergency room with headaches, altered mental status, and a prior presentation of location-related symptomatic epilepsy. Magnetic resonance imaging depicted a mass in the clivus, low in signal on T1 and mildly hypointense on T2 imaging. Follow-up computed tomography (CT) imaging, as recommended, revealed the classic presentation of FDC. In this paper, we discuss the significance of this condition and the importance of thorough investigation to rule out differential diagnoses that may present with similar acute symptoms as this patient.
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Affiliation(s)
- Veronica E Nkie
- Osteopathic Medicine-IV (OMS-IV), Alabama College of Osteopathic Medicine, Alabama, USA
| | - Sandra Martin
- Radiology, Coosa Valley Medical Center, Alabama, USA
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17
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Yoo HD, Chung JC, Park KS, Chung SY, Park MS, Ryu S, Kim SM. Long-Term Outcomes after Multimodal Treatment for Clival Chordoma: Efficacy of the Endonasal Transclival Approach with Early Adjuvant Radiation Therapy. J Clin Med 2023; 12:4460. [PMID: 37445495 DOI: 10.3390/jcm12134460] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2023] [Revised: 06/28/2023] [Accepted: 06/29/2023] [Indexed: 07/15/2023] Open
Abstract
This study investigates the long-term outcomes of clival chordoma patients treated with the endonasal transclival approach (ETCA) and early adjuvant radiation therapy. A retrospective review of 17 patients (2002-2013) showed a 10-year progression-free survival (PFS) rate of 67.4%, with the ETCA group showing fewer progressions and cranial neuropathies than those treated with combined approaches. The ETCA, a minimally invasive technique, provided a similar extent of resection compared to conventional skull-base approaches and enabled safe delivery of high-dose adjuvant radiotherapy. The findings suggest that ETCA is an effective treatment for centrally located clival chordomas.
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Affiliation(s)
- Hyun Dong Yoo
- Department of Neurosurgery, Eulji University Hospital, Eulji University College of Medicine, Daejeon 35233, Republic of Korea
| | - Jong Chul Chung
- Center for Neuromodulation, Department of Neurosurgery, NYU Langone Medical Center, New York, NY 11021, USA
| | - Ki Seok Park
- Department of Neurosurgery, Eulji University Hospital, Eulji University College of Medicine, Daejeon 35233, Republic of Korea
| | - Seung Young Chung
- Department of Neurosurgery, Eulji University Hospital, Eulji University College of Medicine, Daejeon 35233, Republic of Korea
| | - Moon Sun Park
- Department of Neurosurgery, Eulji University Hospital, Eulji University College of Medicine, Daejeon 35233, Republic of Korea
| | - Seungjun Ryu
- Department of Neurosurgery, Eulji University Hospital, Eulji University College of Medicine, Daejeon 35233, Republic of Korea
| | - Seong Min Kim
- Department of Neurosurgery, Eulji University Hospital, Eulji University College of Medicine, Daejeon 35233, Republic of Korea
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18
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Battal B, Zamora C. Imaging of Skull Base Tumors. Tomography 2023; 9:1196-1235. [PMID: 37489465 PMCID: PMC10366931 DOI: 10.3390/tomography9040097] [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: 05/19/2023] [Revised: 06/19/2023] [Accepted: 06/19/2023] [Indexed: 07/26/2023] Open
Abstract
The skull base provides a platform for supporting the brain while serving as a conduit for major neurovascular structures. In addition to malignant lesions originating in the skull base, there are many benign entities and developmental variants that may simulate disease. Therefore, a basic understanding of the relevant embryology is essential. Lesions centered in the skull base can extend to the adjacent intracranial and extracranial compartments; conversely, the skull base can be secondarily involved by primary extracranial and intracranial disease. CT and MRI are the mainstay imaging methods and are complementary in the evaluation of skull base lesions. Advances in cross-sectional imaging have been crucial in the management of patients with skull base pathology, as this represents a complex anatomical area that is hidden from direct clinical exam. Furthermore, the clinician must rely on imaging studies for therapy planning and to monitor treatment response. It is crucial to have a thorough understanding of skull base anatomy and its various pathologies, as well as to recognize the appearance of treatment-related changes. In this review, we aim to describe skull base tumors and tumor-like lesions in an anatomical compartmental approach and present imaging methods that aid in diagnosis, management, and follow-up.
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Affiliation(s)
- Bilal Battal
- Division of Neuroradiology, Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, NC 27599, USA
| | - Carlos Zamora
- Division of Neuroradiology, Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, NC 27599, USA
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19
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Ito N, Fujii H, Kuroda R, Matsuki M, Mori H. Intradural Chordoma Mimicking an Epidermoid Cyst on Imaging. Cureus 2023; 15:e40610. [PMID: 37476114 PMCID: PMC10354377 DOI: 10.7759/cureus.40610] [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: 06/18/2023] [Indexed: 07/22/2023] Open
Abstract
Chordomas are rare, locally aggressive, primary bone tumors derived from primitive notochord remnants. They almost always arise within the axial skeleton, particularly in the skull base and the sacrococcygeal region. They usually present as extradural tumors, but rarely, they present as entirely intradural tumors. This report describes a case of intradural chordoma that mimicked an epidermoid cyst. A 72-year-old woman was incidentally found to have a prepontine extra-axial mass on magnetic resonance imaging. The mass gradually increased in size, and she felt discomfort in the right cheek area. The mass showed similar signal intensity to cerebrospinal fluid on T1-weighted images and T2-weighted images, but high signal intensity on fluid-attenuated inversion recovery images and diffusion-weighted images. Because the presence of very faint contrast enhancement was not noticed, the mass was preoperatively diagnosed as an epidermoid cyst. Tumor resection was performed, and the histopathological diagnosis was chondroid chordoma. Since intradural chordoma may resemble an epidermoid cyst on imaging, radiologists should check carefully for the presence of contrast enhancement and suggest the possibility of intradural chordoma.
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Affiliation(s)
- Noriko Ito
- Radiology, Jichi Medical University, School of Medicine, Tochigi, JPN
| | - Hiroyuki Fujii
- Radiology, Jichi Medical University, School of Medicine, Tochigi, JPN
| | - Rintaro Kuroda
- Neurological Surgery, Jichi Medical University, School of Medicine, Tochigi, JPN
| | - Mitsuru Matsuki
- Radiology, Jichi Children's Medical Center Tochigi, Tochigi, JPN
| | - Harushi Mori
- Radiology, Jichi Medical University, School of Medicine, Tochigi, JPN
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20
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Sethi KS, Choudhary S, Ganesan PK, Sood N, Ramalingum WBS, Basil R, Dhawan S. Sphenoid sinus anatomical variants and pathologies: pictorial essay. Neuroradiology 2023:10.1007/s00234-023-03163-4. [PMID: 37202536 DOI: 10.1007/s00234-023-03163-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/02/2023] [Accepted: 05/10/2023] [Indexed: 05/20/2023]
Abstract
The sphenoid sinus (SS) is one of the four paired paranasal sinuses (PNSs) within the sphenoid bone body. Isolated pathologies of sphenoid sinus are uncommon. The patient may have various presentations like headache, nasal discharge, post nasal drip, or non-specific symptoms. Although rare, potential complications of sphenoidal sinusitis can range from mucocele to skull base or cavernous sinus involvement, or cranial neuropathy. Primary tumors are rare and adjoining tumors secondarily invading the sphenoid sinus is seen. Multidetector computed tomography (CT) scan and magnetic resonance imaging (MRI) are the primary imaging modalities used to diagnose various forms of sphenoid sinus lesions and complications. We have compiled anatomic variants and various pathologies affecting sphenoid sinus lesions in this article.
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Affiliation(s)
- Kanika Sekhri Sethi
- Department of Radiodiagnosis, Dr. B. L. Kapur Memorial Hospital, Pusa Road, New Delhi, 110 005, India.
| | - Subham Choudhary
- Department of Radiodiagnosis, Dr. B. L. Kapur Memorial Hospital, Pusa Road, New Delhi, 110 005, India
| | - Prem Kumar Ganesan
- Department of Radiodiagnosis, Dr. B. L. Kapur Memorial Hospital, Pusa Road, New Delhi, 110 005, India
| | - Neha Sood
- Department of ENT, Dr. B. L. Kapur Memorial Hospital, Pusa Road, New Delhi, 110 005, India
| | - W B S Ramalingum
- Department of ENT, Dr. B. L. Kapur Memorial Hospital, Pusa Road, New Delhi, 110 005, India
| | - Rohit Basil
- Department of Neurosurgery, Dr. B. L. Kapur Memorial Hospital, Pusa Road, New Delhi, 110 005, India
| | - Sugandha Dhawan
- St. Stephen's Hospital, Tis Hazari, New Delhi, 110 054, India
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21
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Wogram E, Schlunk F, Shah MJ, Prinz M, Urbach H, Erny D, Taschner CA. Freiburg Neuropathology Case Conference : A 51-year-old Patient Presenting with Epistaxis and Occasional Headaches 16 Years after Diagnosis of a Grade 1 Chondrosarcoma of the Left Petrous Apex. Clin Neuroradiol 2023; 33:569-575. [PMID: 37171609 DOI: 10.1007/s00062-023-01294-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/17/2023] [Indexed: 05/13/2023]
Affiliation(s)
- E Wogram
- Department of Neuropathology, Medical Centre, University of Freiburg, Freiburg, Germany
- Faculty of Medicine, Medical Centre, University of Freiburg, Freiburg, Germany
| | - F Schlunk
- Department of Neuroradiology, Medical Centre, University of Freiburg, Breisacherstraße 64, 79106, Freiburg, Germany
- Faculty of Medicine, Medical Centre, University of Freiburg, Freiburg, Germany
| | - M J Shah
- Department of Neurosurgery, Medical Centre, University of Freiburg, Freiburg, Germany
- Faculty of Medicine, Medical Centre, University of Freiburg, Freiburg, Germany
| | - M Prinz
- Department of Neuropathology, Medical Centre, University of Freiburg, Freiburg, Germany
- Faculty of Medicine, Medical Centre, University of Freiburg, Freiburg, Germany
| | - H Urbach
- Department of Neuroradiology, Medical Centre, University of Freiburg, Breisacherstraße 64, 79106, Freiburg, Germany
- Faculty of Medicine, Medical Centre, University of Freiburg, Freiburg, Germany
| | - D Erny
- Department of Neuropathology, Medical Centre, University of Freiburg, Freiburg, Germany
- Faculty of Medicine, Medical Centre, University of Freiburg, Freiburg, Germany
| | - C A Taschner
- Department of Neuroradiology, Medical Centre, University of Freiburg, Breisacherstraße 64, 79106, Freiburg, Germany.
- Faculty of Medicine, Medical Centre, University of Freiburg, Freiburg, Germany.
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22
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Supsupin EP, Gonzales NS, Debnam JM. Anatomy and Pathology of the Skull Base: Malignant and Nonmalignant Lesions. Oral Maxillofac Surg Clin North Am 2023:S1042-3699(23)00025-0. [PMID: 37142448 DOI: 10.1016/j.coms.2023.03.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/06/2023]
Abstract
The skull base (SB) is the osseous foundation of the cranial vault. It contains many openings that allow communication between the extracranial and intracranial structures. This communication is crucial in normal physiologic processes yet may also arrow spread of disease. This article provides a comprehensive review of SB anatomy including important landmarks and anatomic variants relevant to SB surgery. We also illustrate the diverse pathologies affecting the SB.
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Affiliation(s)
- Emilio P Supsupin
- Radiology/Neuroradiology, Radiology Residency Program, University of Florida College of Medicine, 655 West 8th. Street, Jacksonville, FL 32209, USA.
| | - Noelani S Gonzales
- Nova Southeastern University, Dr. Kiran C. Patel College of Osteopathic Medicine, Fort Lauderdale, FL 33314, USA
| | - James Matthew Debnam
- Neuroradiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
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23
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Cho HJ, Hahm MH, Kim JS, Heo SJ. Arrested Pneumatization of the Sphenoid Sinus. J Craniofac Surg 2023; 34:e246-e247. [PMID: 36730881 DOI: 10.1097/scs.0000000000009097] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/14/2022] [Accepted: 09/04/2022] [Indexed: 02/04/2023] Open
Abstract
The sphenoid bone is a complex structure in terms of its embryological origin. At birth, the sphenoid sinus is nonpneumatized. Arrested pneumatization of the sphenoid sinus is considered a normal anatomic variant but may be mistaken for disease in imaging studies. We report 2 cases of arrested pneumatization of the sphenoid sinus, a normal variant commonly misdiagnosed as a serious disease of the skull base. A 29-year-old man with a complaint of dizziness visited a local clinic for assessment. Computed tomography (CT) of the paranasal sinuses (PNS) showed a noneroding, nonexpansile, and nonhomogenous lesion in the sphenoid bone. Magnetic resonance imaging (MRI) of the brain revealed a high-signal lesion on both T1-weighted and T2-weighted images. Given these typical findings in the CT of PNS and MRI of the brain, the lesion was diagnosed as arrested pneumatization of the sphenoid sinus. In the second case, a 60-year-old woman with a complaint of headache visited a local clinic for assessment. CT of PNS showed a fibro-osseous lesion (with features of sclerosis and osteolysis) in the skull base. Brain MRI revealed a low-signal lesion on a T1-weighted image containing a high-signal intensity around the sphenoid bone, thereby suggesting internal fat contents. A precise interpretation of CT of PNS and brain MRI is essential to distinguish arrested pneumatization of the sphenoid sinus and to help establish a differential diagnosis and avoid needless biopsy.
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Affiliation(s)
| | - Myong Hun Hahm
- Radiology, School of Medicine, Kyungpook National University, Daegu, South Korea
| | - Jung Soo Kim
- Department of Otorhinolaryngology-Head and Neck Surgery
| | - Sung Jae Heo
- Department of Otorhinolaryngology-Head and Neck Surgery
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24
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Gbadamosi WA, Knezevic-Maragh M, Talukder P, Smith W, Sanchez EZ. Pre-resection Embolization of a Focal Lumbar Chordoma. Cureus 2023; 15:e38406. [PMID: 37265900 PMCID: PMC10231904 DOI: 10.7759/cureus.38406] [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: 05/01/2023] [Indexed: 06/03/2023] Open
Abstract
Chordoma is a slow-growing local invasive tumor with high mortality and recurrence rates after surgical resection. It can affect the clivus and sacrum and rarely involve the lumbar vertebra. There is limited literature research describing lumbar embolization before surgical resection in lumbar chordoma. Thus, this case report describes an atypical patient with chronic lower back pain who presented to the hospital for worsening pain. Radiological images show an aggressive focal lesion at the second lumbar spine extending into the posterior element. The patient underwent lumbar artery embolization before surgical resection. The final pathology diagnosis confirmed a conventional chordoma. Therefore, patients with radiological imaging features of conventional chordoma may benefit from embolization prior to surgical resection to decrease intraoperative bleeding.
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Affiliation(s)
| | | | - Priotush Talukder
- College of Medicine, American University of Antigua, St. John's, ATG
| | - Weber Smith
- Pathology and Laboratory Medicine, Medical Center of Trinity, Trinity, USA
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25
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Ottenhausen M, Greco E, Bertolini G, Gerosa A, Ippolito S, Middlebrooks EH, Serrao G, Bruzzone MG, Costa F, Ferroli P, La Corte E. Craniovertebral Junction Instability after Oncological Resection: A Narrative Review. Diagnostics (Basel) 2023; 13:1502. [PMID: 37189602 PMCID: PMC10137736 DOI: 10.3390/diagnostics13081502] [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: 02/27/2023] [Revised: 04/16/2023] [Accepted: 04/19/2023] [Indexed: 05/17/2023] Open
Abstract
The craniovertebral junction (CVJ) is a complex transition area between the skull and cervical spine. Pathologies such as chordoma, chondrosarcoma and aneurysmal bone cysts may be encountered in this anatomical area and may predispose individuals to joint instability. An adequate clinical and radiological assessment is mandatory to predict any postoperative instability and the need for fixation. There is no common consensus on the need for, timing and setting of craniovertebral fixation techniques after a craniovertebral oncological surgery. The aim of the present review is to summarize the anatomy, biomechanics and pathology of the craniovertebral junction and to describe the available surgical approaches to and considerations of joint instability after craniovertebral tumor resections. Although a one-size-fits-all approach cannot encompass the extremely challenging pathologies encountered in the CVJ area, including the possible mechanical instability that is a consequence of oncological resections, the optimal surgical strategy (anterior vs posterior vs posterolateral) tailored to the patient's needs can be assessed preoperatively in many instances. Preserving the intrinsic and extrinsic ligaments, principally the transverse ligament, and the bony structures, namely the C1 anterior arch and occipital condyle, ensures spinal stability in most of the cases. Conversely, in situations that require the removal of those structures, or in cases where they are disrupted by the tumor, a thorough clinical and radiological assessment is needed to timely detect any instability and to plan a surgical stabilization procedure. We hope that this review will help shed light on the current evidence and pave the way for future studies on this topic.
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Affiliation(s)
- Malte Ottenhausen
- Department of Neurological Surgery, University Medical Center Mainz, 55131 Mainz, Germany
| | - Elena Greco
- Department of Radiology, Mayo Clinic, Jacksonville, FL 32224, USA
| | - Giacomo Bertolini
- Head and Neck Department, Neurosurgery Division, Azienda Ospedaliero-Universitaria di Parma, 43126 Parma, Italy
| | - Andrea Gerosa
- Head and Neck Department, Neurosurgery Division, Azienda Ospedaliero-Universitaria di Parma, 43126 Parma, Italy
| | - Salvatore Ippolito
- Head and Neck Department, Neurosurgery Division, Azienda Ospedaliero-Universitaria di Parma, 43126 Parma, Italy
| | - Erik H. Middlebrooks
- Department of Radiology, Mayo Clinic, Jacksonville, FL 32224, USA
- Department of Neurosurgery, Mayo Clinic, Jacksonville, FL 32224, USA
| | - Graziano Serrao
- Department of Health Sciences, San Paolo Medical School, Università Degli Studi di Milano, 20142 Milan, Italy
| | - Maria Grazia Bruzzone
- Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy
| | - Francesco Costa
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy
| | - Paolo Ferroli
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy
| | - Emanuele La Corte
- Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milan, Italy
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26
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Alsavaf MB, Prevedello DM. Letter: Chordoma and Ecchordosis Physaliphora: Two Sides of the Same Coin. Neurosurgery 2023; 92:e116-e117. [PMID: 36877027 DOI: 10.1227/neu.0000000000002451] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2023] [Accepted: 01/18/2023] [Indexed: 03/07/2023] Open
Affiliation(s)
- Mohammad Bilal Alsavaf
- Department of Otolaryngology-Head and Neck Surgery, Wexner Medical Center at The Ohio State University, Columbus, Ohio, USA.,Department of Neurological Surgery, Wexner Medical Center at The Ohio State University, Columbus, Ohio, USA
| | - Daniel M Prevedello
- Department of Otolaryngology-Head and Neck Surgery, Wexner Medical Center at The Ohio State University, Columbus, Ohio, USA.,Department of Neurological Surgery, Wexner Medical Center at The Ohio State University, Columbus, Ohio, USA
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27
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Palsetia DR, Vijan AV, Gala FB, Sahu AC, Patkar DP, A. AS. Clival and Paraclival Lesions: A Pictorial Review. Indian J Radiol Imaging 2023; 33:201-217. [PMID: 37123565 PMCID: PMC10132890 DOI: 10.1055/s-0043-1761183] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/13/2023] Open
Abstract
AbstractThe clivus is a midline anatomical structure in the central skull base. It is affected by a wide range of non-neoplastic, benign and malignant pathologies, some of which typically affect the clivus because of its strategic location and embryological origins. Clival lesions may often be asymptomatic with occasional complaints like headache or cranial neuropathy in few. Cross-sectional imaging techniques, namely, computed tomographic scan and magnetic resonance imaging, thus, play a key role in approximating to the final diagnosis and estimating the disease extent. In this article, we highlight the important imaging features of various clival and paraclival pathologies to facilitate effective diagnosis, therapeutic planning, and management.
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Affiliation(s)
- Delnaz R. Palsetia
- Department of Radiodiagnosis and Imaging, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
| | - Antariksh V. Vijan
- Department of Radiodiagnosis and Imaging, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
| | - Foram B. Gala
- Department of Radiodiagnosis and Imaging, Lifescan Imaging Centre & Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
| | - Amit C. Sahu
- Department of Interventional Neuro-Radiology, Wockhardt Hospital, Mumbai, Maharashtra, India
| | - Deepak P. Patkar
- Department of Imaging, Nanavati Superspecialty Hospital, Mumbai, Maharashtra, India
| | - Arpita Sahu A.
- Department of Radiodiagnosis and Imaging, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
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28
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Kesari S, Williams J, Burbano E, Stirn M, Caroen S, Oronsky B, Reid T, Larson C. Case Report of AdAPT-001-Mediated Sensitization to a Previously Failed Checkpoint Inhibitor in a Metastatic Chordoma Patient. Case Rep Oncol 2023; 16:172-176. [PMID: 37008834 PMCID: PMC10051040 DOI: 10.1159/000529503] [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: 08/17/2022] [Accepted: 01/27/2023] [Indexed: 03/30/2023] Open
Abstract
Chordoma is a rare, but aggressive bone tumor with a high recurrence rate that primarily arises at the cranial and caudal ends of the axial skeleton. Systemic chemotherapies are not effective against the tumor, and outside of surgical resection and radiation, no approved options are available. Prognosis depends on the extent of surgical resection, with the more the better, and adjuvant radiotherapy. Herein is presented the first-ever case of a recurrent chordoma patient that responded to the combination of one dose of an experimental TGF-beta trap carrying oncolytic adenovirus, known as AdAPT-001, followed by immune checkpoint inhibitor therapy, despite prior progression on an anti-PD-1. This case report highlights the potential of AdAPT-001 as a treatment modality in combination with checkpoint inhibition for recurrent chordoma.
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Affiliation(s)
- Santosh Kesari
- Pacific Neuroscience Institute and Saint John’s Cancer Institute at Providence Saint John’s Health Center, Santa Monica, CA, USA
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29
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Hu YS, Lee CC, Wu CA, Lin CJ, Yang HC, Guo WY, Liu KD, Chung WY, Shiau CY, Wu HM. Magnetic resonance imaging signal characteristics associated with prognosis of skull base chordoma after gamma knife radiosurgery. J Neurooncol 2023; 161:45-56. [PMID: 36565364 DOI: 10.1007/s11060-022-04199-x] [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: 10/14/2022] [Accepted: 11/14/2022] [Indexed: 12/25/2022]
Abstract
OBJECTIVE To investigate the association between the magnetic resonance imaging (MRI) signal characteristics of skull base chordoma and radiosurgical outcomes. METHODS Twenty-four patients with skull base chordomas treated with Gamma Knife radiosurgery (GKRS) after previous surgical resection were retrospectively (2001-2021) examined. Pre-GKRS MRIs were analyzed for RT2 (tumor-to-brainstem signal intensity ratio on T2-weighted imaging), RCE (tumor-to-brainstem signal intensity ratio on contrast-enhanced T1-weighted imaging), and mean apparent diffusion coefficient (ADC). Correlations of the parameters with patient survival and local tumor progression were made by using Cox regression and Kaplan-Meier analyses. RESULTS During a median follow-up of 46 months after GKRS, 9 patients died with significantly more local tumor progression events (median number: 2 vs 0, P = .012) than did 15 alive patients. On multivariable analysis, higher mean ADC was associated with longer patient survival (P = .016) after GKRS. The actuarial 5-year overall survival rates were 88.9% versus 54.7% for chordomas with an ADC of ≥ 1270 × 10-6 mm2/s versus < 1270 × 10-6 mm2/s. RT2 < 1.5 (P = .038) and RCE > 1.57 (P = .022) were associated with a lower probability of local tumor control. CONCLUSION Lower mean ADC values are associated with shorter patient survival in skull base chordomas after GKRS. Diffusion-weighted imaging may help in GKRS planning and outcome prediction for these patients.
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Affiliation(s)
- Yong-Sin Hu
- Department of Radiology, Taipei Hospital, Ministry of Health and Welfare, New Taipei, Taiwan.,School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Radiology, Taipei Veterans General Hospital, No. 201, Shipai Rd., Sec. 2, Beitou District, Taipei, 112, Taiwan
| | - Cheng-Chia Lee
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Chia-An Wu
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Radiology, Taipei Veterans General Hospital, No. 201, Shipai Rd., Sec. 2, Beitou District, Taipei, 112, Taiwan.,Department of Radiology, Shuang Ho Hospital, Taipei Medical University, Taipei, Taiwan
| | - Chung-Jung Lin
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Radiology, Taipei Veterans General Hospital, No. 201, Shipai Rd., Sec. 2, Beitou District, Taipei, 112, Taiwan
| | - Huai-Che Yang
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Wan-Yuo Guo
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Radiology, Taipei Veterans General Hospital, No. 201, Shipai Rd., Sec. 2, Beitou District, Taipei, 112, Taiwan
| | - Kang-Du Liu
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Wen-Yuh Chung
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan.,Department of Neurosurgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
| | - Cheng-Ying Shiau
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.,Cancer Center, Taipei Veterans General Hospital, Taipei, Taiwan
| | - Hsiu-Mei Wu
- School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan. .,Department of Radiology, Taipei Veterans General Hospital, No. 201, Shipai Rd., Sec. 2, Beitou District, Taipei, 112, Taiwan.
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30
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Fiani B, Jarrah R, Shields J, Durrani S, Panico N, Mualem W, Nathani KR, Pasko K. A Comprehensive Overview of Pediatric Neoplasms at the Craniocervical Junction: Meningiomas, Schwannomas, and Chordomas. Cureus 2022; 14:e31083. [DOI: 10.7759/cureus.31083] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2022] [Accepted: 11/04/2022] [Indexed: 11/06/2022] Open
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31
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Lee SH, Kwok KY, Wong SM, Chan CXJ, Wong YT, Tsang ML. Chordoma at the skull base, spine, and sacrum: A pictorial essay. J Clin Imaging Sci 2022; 12:44. [PMID: 36128361 PMCID: PMC9479632 DOI: 10.25259/jcis_62_2022] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/05/2022] [Accepted: 07/10/2022] [Indexed: 11/04/2022] Open
Abstract
Chordomas are rare tumors believed to be arising from the notochord remnant in the axial skeleton. Diagnosis is often difficult since they show overlapping imaging features with other more common disease including metastases. Since individualized papers are only discussing the imaging features at different locations, the aim of this pictorial review is to have a comprehensive review on the common imaging findings of chordomas along the entire neuroaxis with a series of pathological proven cases in a local tertiary hospital in Hong Kong.
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Affiliation(s)
- Sin Hang Lee
- Department of Radiology, Tuen Mun Hospital, Tuen Mun, Hong Kong,
| | - Kai Yan Kwok
- Department of Radiology, Tuen Mun Hospital, Tuen Mun, Hong Kong,
| | - Sin Man Wong
- Department of Radiology, CUHK Medical Centre, Sha Tin, Hong Kong,
| | | | - Yu Ting Wong
- Department of Radiology, Tuen Mun Hospital, Tuen Mun, Hong Kong,
| | - Man Lung Tsang
- Department of Radiology, Tuen Mun Hospital, Tuen Mun, Hong Kong,
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32
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Nardi C, Maraghelli D, Pietragalla M, Scola E, Locatello LG, Maggiore G, Gallo O, Bartolucci M. A practical overview of CT and MRI features of developmental, inflammatory, and neoplastic lesions of the sphenoid body and clivus. Neuroradiology 2022; 64:1483-1509. [PMID: 35657394 PMCID: PMC9271108 DOI: 10.1007/s00234-022-02986-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/22/2021] [Accepted: 05/18/2022] [Indexed: 11/28/2022]
Abstract
The sphenoid bone is an unpaired bone that contributes to the formation of the skull base. Despite the enormous progress in transnasal endoscopic visualisation, imaging techniques remain the cornerstones to characterise any pathological condition arising in this area. In the present review, we offer a bird's-eye view of the developmental, inflammatory, and neoplastic alterations affecting the sphenoid body and clivus, with the aim to propose a practical diagnostic aid for radiologists based on clinico-epidemiological, computed tomography, and magnetic resonance imaging features.
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Affiliation(s)
- Cosimo Nardi
- Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit N. 2, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla 3, 50134 Florence, Italy
| | - Davide Maraghelli
- Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit N. 2, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla 3, 50134 Florence, Italy
| | - Michele Pietragalla
- Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit N. 2, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla 3, 50134 Florence, Italy
| | - Elisa Scola
- Department of Neuroradiology, Careggi University Hospital, Largo Piero Palagi 1, 50134 Florence, Italy
| | - Luca Giovanni Locatello
- Department of Otorhinolaryngology, Careggi University Hospital, Via Taddeo Alderotti, 50139 Florence, Italy
| | - Giandomenico Maggiore
- Department of Otorhinolaryngology, Careggi University Hospital, Via Taddeo Alderotti, 50139 Florence, Italy
| | - Oreste Gallo
- Department of Otorhinolaryngology, Careggi University Hospital, Via Taddeo Alderotti, 50139 Florence, Italy
- Department of Experimental and Clinical Medicine, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla 3, 50134 Florence, Italy
| | - Maurizio Bartolucci
- Department of Radiology, Azienda USL Toscana Centro, Santo Stefano Hospital, Via Suor Niccolina Infermiera, 20/22, 59100 Prato, Italy
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33
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Rao D, Stein R, Jenson M, Patel J, Fiester P. Imaging Review of the Endoscopic Operative Corridor for Clivus Lesions. Curr Probl Diagn Radiol 2022; 52:66-76. [DOI: 10.1067/j.cpradiol.2022.07.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2022] [Revised: 06/09/2022] [Accepted: 07/27/2022] [Indexed: 11/22/2022]
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Hua JJ, Ying ML, Chen ZW, Huang C, Zheng CS, Wang YJ. Chordoma of petrosal mastoid region: A case report. World J Clin Cases 2022; 10:5331-5336. [PMID: 35812686 PMCID: PMC9210881 DOI: 10.12998/wjcc.v10.i16.5331] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2021] [Revised: 10/04/2021] [Accepted: 04/21/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Chordoma is a rare low-grade malignant tumor originating from embryonic notochordal tissue mainly occurring in the axial bone, mostly in the spheno-occipital junction and sacrococcyx, which accounts for approximately 1% of all malignant bone tumors and 0.1%–0.2% of intracranial tumors. Chordoma in the petrous mastoid region is rare.
CASE SUMMARY We describe a 36-year-old male patient with chordoma in the left petrous mastoid region. The main clinical manifestations were pain and discomfort, which lasted for 2 years. Magnetic resonance imaging showed a lobulated mass in the left petrous mastoid with an unclear boundary and obvious enhancement. The tumor was completely removed after surgical treatment, and a histological examination confirmed that the tumor was a chordoma. During 5 years of follow-up, no clinical or radiological evidence of recurrence or metastasis was found.
CONCLUSION Chordoma in the petrosal mastoid region is rare but should be included in differential diagnosis of petrosal mastoid tumors.
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Affiliation(s)
- Jian-Jun Hua
- Department of Radiology, Jinhua Municipal Central Hospital, Jinhua 321000, Zhejiang Province, China
| | - Ming-Liang Ying
- Department of Radiology, Jinhua Municipal Central Hospital, Jinhua 321000, Zhejiang Province, China
| | - Zhen-Wei Chen
- Department of Pathology, Jinhua Municipal Central Hospita, Jinhua 321000, Zhejiang Province, China
| | - Cong Huang
- Department of Radiology, No. 926 Hospital, Joint Logistics Support Force of PLA, Kaiyuan 616000, Yunnan Province, China
| | - Chu-Shan Zheng
- Department of Radiology, Sun Yat-sen Memorial Hospital Sun Yat-Sen University, Guangzhou 51000, Guangdong Province, China
| | - Yu-Jun Wang
- Department of Radiology, Zhejiang Provincial Hospital of Chinese Medicine, Hangzhou 310000, Zhejiang Province, China
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35
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Hazra D, Balasubramanian C, Das S, Tiwari M, Ghosh A. A Rare Case Report of an Intradural Left Cerebellopontine Angle Chordoma. Asian J Neurosurg 2022; 17:134-136. [PMID: 35873840 PMCID: PMC9298575 DOI: 10.1055/s-0042-1749128] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
Abstract
AbstractIntracranial intradural chordomas are rare entities constituting 1 to 3% of primary bone tumors. The mainstay of treatment remains aggressive resection of the lesion followed by adjuvant radiation therapy. We hereby report a case of a 70-year-old gentleman with intracranial, intradural chordoma arising from the left cerebellopontine angle. We hope to add to the existing minimal literature on this subject by highlighting this case, the first reported one from Asia.
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Affiliation(s)
- Darpanarayan Hazra
- Department of Emergency Medicine, Institute of Neuroscience Kolkata, Kolkata, West Bengal, India
| | | | - Soutrik Das
- Department of Neuropathology, Institute of Neuroscience Kolkata, Kolkata, West Bengal, India
| | - Mona Tiwari
- Department of Neuroradiology, Institute of Neuroscience Kolkata, Kolkata, West Bengal, India
| | - Amit Ghosh
- Department of Neurosurgery, Institute of Neuroscience Kolkata, Kolkata, West Bengal, India
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36
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Lubomirsky B, Jenner ZB, Jude MB, Shahlaie K, Assadsangabi R, Ivanovic V. Sellar, suprasellar, and parasellar masses: Imaging features and neurosurgical approaches. Neuroradiol J 2022; 35:269-283. [PMID: 34856828 PMCID: PMC9244752 DOI: 10.1177/19714009211055195] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
The sellar, suprasellar, and parasellar space contain a vast array of pathologies, including neoplastic, congenital, vascular, inflammatory, and infectious etiologies. Symptoms, if present, include a combination of headache, eye pain, ophthalmoplegia, visual field deficits, cranial neuropathy, and endocrine manifestations. A special focus is paid to key features on CT and MRI that can help in differentiating different pathologies. While most lesions ultimately require histopathologic evaluation, expert knowledge of skull base anatomy in combination with awareness of key imaging features can be useful in limiting the differential diagnosis and guiding management. Surgical techniques, including endoscopic endonasal and transcranial neurosurgical approaches are described in detail.
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Affiliation(s)
- Bryan Lubomirsky
- Department of Radiology, Section of
Neuroradiology, University of California Davis
Medical Center, Sacramento, CA, USA
| | - Zachary B Jenner
- Department of Radiology, Diagnostic
and Interventional Radiology Residency, University of California Davis
Medical Center, Sacramento, CA, USA
| | - Morgan B Jude
- School of Medicine, University of California Davis
Medical Center, Sacramento, CA, USA
| | - Kiarash Shahlaie
- Department of Neurological Surgery, University of California Davis
Medical Center, Sacramento, CA, USA
| | - Reza Assadsangabi
- Department of Radiology, Section of
Neuroradiology, University of California Davis
Medical Center, Sacramento, CA, USA
| | - Vladimir Ivanovic
- Department of Radiology, Section of
Neuroradiology, University of California Davis
Medical Center, Sacramento, CA, USA
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37
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Mark IT, Van Gompel JJ, Inwards CY, Ball MK, Morris JM, Carr CM. MRI enhancement patterns in 28 cases of clival chordomas. J Clin Neurosci 2022; 99:117-122. [PMID: 35278932 DOI: 10.1016/j.jocn.2022.02.037] [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: 01/26/2021] [Revised: 02/09/2022] [Accepted: 02/27/2022] [Indexed: 10/18/2022]
Abstract
Clival chordomas are classically thought of as locally aggressive tumors of the skull base and differentiate themselves from their benign counterparts by demonstrating moderate to marked contrast enhancement, reported as 95-100% in prior studies. The purpose of this review was to evaluate the imaging characteristics of lesions from a single institution classified as clival chordomas with an emphasis of highlighting lesions that do not follow the prevalent current description for chordoma. We searched our institutional databases for all patients with pathologically proven clival chordomas from 1997 to 2017 who had pre-operative imaging available. The images were evaluated for degree of contrast enhancement, MRI signal characteristics, osseous involvement, location, aggressiveness of appearance, and presence of calcifications. 28 cases were identified that had preoperative imaging available for review. Over half of the patients demonstrated either no/minimal (11/28, 39%) or mild enhancement (7/28, 25%). The remaining cases demonstrated moderate (4/28, 14%) and marked enhancement (6/28, 21%). The 4 lesions measuring less than 20 mm all had mild to minimal/no enhancement and lacked aggressive features on CT. Our experience finds that over half (64%) of clival chordomas will demonstrate mild or no enhancement at all. These findings suggest that the lack of MRI contrast enhancement should not be synonymous with a benign clival mass.
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Affiliation(s)
- Ian T Mark
- Mayo Clinic, Department of Radiology. Rochester, MN, USA.
| | | | | | | | | | - Carrie M Carr
- Mayo Clinic, Department of Radiology. Rochester, MN, USA
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38
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Hall MN, Raviskanthan S, Mortensen PW, Lee AG. Isolated Fourth Nerve Palsy as the Presenting Sign of Clival Chordoma. J Neuroophthalmol 2022; 42:e391-e393. [PMID: 33449491 DOI: 10.1097/wno.0000000000001169] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Affiliation(s)
- MacGregor N Hall
- McGovern Medical School at the University of Texas Health Science Center at Houston (MNH), Houston, Texas ; Department of Ophthalmology (SR, PM, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; and Department of Ophthalmology (AGL), the University of Iowa Hospitals and Clinics, Iowa City, Iowa
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39
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Guadalupi P, Gessi M, Massimi L, Caldarelli M, Gaudino S. A Cystic Clival Chordoma with CT and MRI Unconventional Appearances. Indian J Radiol Imaging 2022; 32:127-131. [PMID: 35722639 PMCID: PMC9200489 DOI: 10.1055/s-0041-1741044] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/29/2022] Open
Abstract
AbstractWe present the first case of clival cystic chordoma with extradural location, transdural transgression, and moderate bone involvement in a 10-year-old girl. Chordoma showed unconventional appearances on computed tomography (CT) and magnetic resonance imaging (MRI), due to cystic components, extradural space location with extensive intradural extension, moderate superficial bone involvement. Surgery confirmed the extradural location and histopathological examination revealed cystic chordoma. MRI and CT findings were not characteristic for a single lesion; differential diagnoses included cystic lesions such as epidermoid and dermoid cyst, ecchordosis physaliphora, and benign notochordal cell tumors.
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Affiliation(s)
- Pamela Guadalupi
- UOC Radiodiagnostica e Neuroradiologia, Dipartimento di Diagnostica per Immagini, Radioterapia, Oncologia ed Ematologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- UOC Radiodiagnostica e Neuroradiologia, Dipartimento di Diagnostica per Immagini, Radioterapia, Oncologia ed Ematologia, Istituto di Radiologia, Università Cattolica del Sacro Cuore, Rome, Italy
| | - Marco Gessi
- Neuropathology Unit, Division of Pathology, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica del Sacro Cuore, Rome, Italy
| | - Luca Massimi
- Pediatric Neurosurgery Unit, Department of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Pediatric Neurosurgery Unit, Department of Neurosurgery, Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Rome, Italy
| | - Massimo Caldarelli
- Pediatric Neurosurgery Unit, Department of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Pediatric Neurosurgery Unit, Department of Neurosurgery, Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Rome, Italy
| | - Simona Gaudino
- UOC Radiodiagnostica e Neuroradiologia, Dipartimento di Diagnostica per Immagini, Radioterapia, Oncologia ed Ematologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
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40
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Multimodal Intraoperative Image-Driven Surgery for Skull Base Chordomas and Chondrosarcomas. Cancers (Basel) 2022; 14:cancers14040966. [PMID: 35205724 PMCID: PMC8870528 DOI: 10.3390/cancers14040966] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2022] [Revised: 02/06/2022] [Accepted: 02/07/2022] [Indexed: 02/04/2023] Open
Abstract
Given the difficulty and importance of achieving maximal resection in chordomas and chondrosarcomas, all available tools offered by modern neurosurgery are to be deployed for planning and resection of these complex lesions. As demonstrated by the review of our series of skull base chordoma and chondrosarcoma resections in the Advanced Multimodality Image-Guided Operating (AMIGO) suite, as well as by the recently published literature, we describe the use of advanced multimodality intraoperative imaging and neuronavigation as pivotal to successful radical resection of these skull base lesions while preventing and managing eventual complications.
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41
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Chen G, Li M, Xu W, Wang X, Feng M, Wang R, Liu X. Surgical Outcomes of Clival Chordoma Through Endoscopic Endonasal Approach: A Single-Center Experience. Front Endocrinol (Lausanne) 2022; 13:800923. [PMID: 35464053 PMCID: PMC9019489 DOI: 10.3389/fendo.2022.800923] [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] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2021] [Accepted: 03/14/2022] [Indexed: 11/13/2022] Open
Abstract
OBJECTIVE Clival chordoma is a locally aggressive tumor with low metastatic potential. In the past decade, endoscopic endonasal approach (EEA) for clival chordoma has had a higher resection rate and a lower morbidity rate than transcranial approaches. Here, we present our initial single-center experience after EEA of clival chordomas. PATIENTS AND METHODS This study retrospectively analyzed 17 consecutive patients with clival chordoma who received EEA in our department between March 2015 and September 2021. The operation was performed by a single surgeon with EEA. The clinical and pathological characteristics were analyzed along with the surgical outcomes and complications. RESULTS A total of 17 consecutive patients with clival chordoma received EEA with a median follow-up of 29.2 months (range 1-79). Gross total resection (GTR) was performed in 7 cases (41%), subtotal resection (STR) in 7 case (41%) and partially resection (PR) in 3 cases (18%). Cerebrospinal fluid leakage occurred in 2 cases (12%) and meningitis developed in 3 patients (18%) which were all successfully treated with intravenous antibiotics without any complications. There were no perioperative deaths or new focal neurological deficits postoperatively. Four in 7 patients with STR have had radiotherapy while the other three chose to be monitored. Till the last follow-up, three patients in STR group who received radiotherapy (3 in 4) had no tumor regrowth, while one in STR group with radiotherapy (1 in 4) showed tumor progression. Two patients in STR group without radiotherapy (2 in 3) showed stable tumor while the left one (1 in 3) showed tumor progression. One patient in the PR group died of tumor progression 2 years postoperation and the other one showed tumor progression and died of lung cancer 1 year postoperation. In addition, 1 in 7 patients with GTR had tumor recurrence in situ after 10 months and developed surgical pathway seeding in the spinal canal in C1 after 16 months. No recurrence occurred in the other 6 cases with GTR during the follow-up. CONCLUSION Although more cases are needed, our case series showed EEA is a safe and reliable method for clival chordoma with high resection rates and low morbidity rates. GTR without tumor residuum would improve the outcome.
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Affiliation(s)
- Ge Chen
- Department of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing, China
- Chinese Pituitary Specialists Congress, Beijing, China
| | - Mingchu Li
- Department of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing, China
- Chinese Pituitary Specialists Congress, Beijing, China
| | - Wenlong Xu
- Department of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing, China
- Chinese Pituitary Specialists Congress, Beijing, China
| | - Xu Wang
- Department of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing, China
- Chinese Pituitary Specialists Congress, Beijing, China
| | - Ming Feng
- Chinese Pituitary Specialists Congress, Beijing, China
- Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Renzhi Wang
- Chinese Pituitary Specialists Congress, Beijing, China
- Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiaohai Liu
- Department of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing, China
- Chinese Pituitary Specialists Congress, Beijing, China
- *Correspondence: Xiaohai Liu,
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42
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Capillary hemangioma of the sphenoid sinus with intrasellar and parasellar extensions. Radiol Case Rep 2021; 17:156-160. [PMID: 34820041 PMCID: PMC8599932 DOI: 10.1016/j.radcr.2021.10.011] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2021] [Revised: 10/06/2021] [Accepted: 10/07/2021] [Indexed: 11/24/2022] Open
Abstract
Capillary hemangiomas are benign vascular tumors usually diagnosed in infancy and involving skin and soft tissues. We report a rare case of an adult capillary hemangioma of the left sphenoid sinus extending into the intrasellar and parasellar regions. A 71-year-old woman presented with complaints of headaches and diplopia. Magnetic resonance imaging revealed a T2-hyperintense mass-like lesion involving the left posterior ethmoid and sphenoid sinuses and left cavernous sinus, that had extended into the intrasellar and parasellar regions. Dynamic contrast-enhanced computed tomography revealed enhancing vessel-like structures within the lesion during the arterial phase, which were observed to expand further in the late phase of the examination. This characteristic feature indicated increased vascularity, suggesting the presence of a capillary hemangioma. The patient underwent endoscopic resection via the endonasal transpterygoid approach, and the lesion was diagnosed histopathologically as a capillary hemangioma. To aid diagnosis and clinical management, radiologists should be aware of the imaging findings related to capillary hemangiomas.
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43
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Kayahara T, Kurita H, Irie K, Nakahara I, Sasaki T. Intracranial chondrosarcoma located in the region of the posterior clinoid process: a case report. Radiol Case Rep 2021; 17:115-118. [PMID: 34777675 PMCID: PMC8577103 DOI: 10.1016/j.radcr.2021.09.058] [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: 09/24/2021] [Accepted: 09/29/2021] [Indexed: 11/30/2022] Open
Abstract
Intracranial chondrosarcomas located in the region of the posterior clinoid process have not been focused on. Here, we report the case of a 29-year-old woman with a skull base tumor in that region. Seven years after the diagnosis, the tumor had grown and showed calcification and tumor stain; chondrosarcoma, posterior clinoid meningioma, and chordoma were suspected. The patient underwent subtotal tumor resection, and the histopathological study revealed that the tumor was a low-grade chondrosarcoma. Chondrosarcomas can be located in the region of the posterior clinoid process, and not only chordomas but also posterior clinoid meningiomas should be considered as a differential diagnosis of tumors located in that region, especially when the tumor has calcification or receives a vascular supply.
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Affiliation(s)
- Tomomichi Kayahara
- Department of Neurosurgery, Nagoya Kyoritsu Hospital, 1-172 Hokke, Nakagawaku, Nagoya, Aichi 454-0933, Japan
- Corresponding author: T. Kayahara
| | - Hiroki Kurita
- Department of Cerebrovascular Surgery, Saitama Medical University International Medical Center, Hidaka, Saitama, Japan
| | - Keiko Irie
- Department of Neurosurgery, Nagoya Kyoritsu Hospital, 1-172 Hokke, Nakagawaku, Nagoya, Aichi 454-0933, Japan
| | - Ichiro Nakahara
- Department of Comprehensive Strokology, Fujita Health University School of Medicine, Toyoake, Aichi, Japan
| | - Tomio Sasaki
- Department of Neurosurgery, Nagoya Kyoritsu Hospital, 1-172 Hokke, Nakagawaku, Nagoya, Aichi 454-0933, Japan
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44
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Lateral Skull Base Chordoma Mimicking a Paraganglioma. Otol Neurotol 2021; 43:e279-e281. [PMID: 34772882 DOI: 10.1097/mao.0000000000003406] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
We present an unusual case of chordoma arising entirely from the lateral skull base with imaging features suggestive of a paraganglioma. Clinical history, management, histopathology, and imaging characteristics are described, including a review of gallium-dotate PET scanning somatostatinreceptor-positive tumors. We further provide a review of management options, including a summary of our approach with surgical biopsy via retrosigmoid and resection via transtemporal approaches. Based on radiologic characteristics and location, lateral skull base chordoma may arise with isolated lateral skull base involvement and has the potential to be misidentified as a glomus jugulare on initial workup.
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45
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Soule E, Baig S, Fiester P, Holtzman A, Rutenberg M, Tavanaiepour D, Rao D. Current Management and Image Review of Skull Base Chordoma: What the Radiologist Needs to Know. J Clin Imaging Sci 2021; 11:46. [PMID: 34513210 PMCID: PMC8422542 DOI: 10.25259/jcis_139_2021] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2021] [Accepted: 08/14/2021] [Indexed: 11/04/2022] Open
Abstract
Chordomas of the skull-base are typically slow-growing, notochord-derived tumors that most commonly originate along the clivus. Skull base chordoma is treated with surgery and radiotherapy. Local recurrence approaches 50% at 10 years. Radiologists play a critical role in diagnosis, treatment planning, and follow-up. Surgeons and radiation oncologists rely on radiologists for pre-operative delineation of tumor and adjacent anatomy, identification of post-treatment changes and disease recurrence, and radiation treatment effects. This review provides an overview of clinical characteristics, surgical anatomy, indications for radiotherapy, identification of treatment complications, and patterns of disease recurrence for radiologists to provide value in the management of these lesions.
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Affiliation(s)
- Erik Soule
- Department of Neuroradiology, University of Florida College of Medicine, Jacksonville, Florida, United States
| | - Saif Baig
- Department of Radiology, University of Florida College of Medicine, Jacksonville, Florida, United States
| | - Peter Fiester
- Department of Neuroradiology, University of Florida College of Medicine, Jacksonville, Florida, United States
| | - Adam Holtzman
- Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida, United States
| | - Michael Rutenberg
- Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida, United States
| | - Daryoush Tavanaiepour
- Department of Neurosurgery, University of Florida College of Medicine, Jacksonville, Florida, United States
| | - Dinesh Rao
- Department of Neuroradiology, University of Florida College of Medicine, Jacksonville, Florida, United States
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de Almeida GB, Januário G, Carvalho R. Nonenhancing intracranial intradural chordoma mimicking an epidermoid cyst on magnetic resonance imaging: a case report. Radiol Case Rep 2021; 16:2306-2310. [PMID: 34194595 PMCID: PMC8233103 DOI: 10.1016/j.radcr.2021.05.057] [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: 05/11/2021] [Accepted: 05/22/2021] [Indexed: 11/16/2022] Open
Abstract
Chordomas are relatively rare malignant tumors derived from embryonic notochord remnants. Most intracranial chordomas show extradural extension and cause bone erosion. However, a small percentage of these tumors are exclusively intradural and tend to show less aggressive features, although local recurrence and metastatic spread have been described. Intradural chordomas with imaging features similar to epidermoid cysts are exceedingly rare. We describe the case of a nonenhancing and nondestructive intradural prepontine chordoma showing restricted diffusion on magnetic resonance imaging on a 44-year-old man who presented with acute-onset vertigo and vomiting. Subtotal resection of the lesion was performed followed by adjuvant radiation therapy. Histopathological examination revealed a chordoma. This case report highlights the need to include intradural chordomas in the differential diagnosis of a nonenhancing and nondestructive prepontine intradural lesion demonstrating restricted diffusion. Gross total resection, adjuvant radiation therapy in cases of macro/microscopical residual disease and regular follow-up imaging assessment are warranted due to the possibility of local recurrence and metastatic dissemination.
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Affiliation(s)
| | - Gonçalo Januário
- Department of Neurosurgery, Hospital de São José, 1150-199 Lisbon, Portugal
| | - Rui Carvalho
- Department of Neuroradiology, Hospital de São José, 1150-199 Lisbon, Portugal
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Kwon JE, Ji SY, Hwang K, Lee KS, Choe G, Kim CY, Han JH. Management challenges associated with a pineal region chordoma: illustrative case. JOURNAL OF NEUROSURGERY: CASE LESSONS 2021; 1:CASE21110. [PMID: 35855099 PMCID: PMC9245841 DOI: 10.3171/case21110] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/11/2021] [Accepted: 04/26/2021] [Indexed: 11/06/2022]
Abstract
BACKGROUND
Chordomas, which are rare malignant neoplasms arising from notochordal remnants, often cause gradually progressive clinical symptoms. Intradural cranial chordomas (ICCs) are extremely rare and generally have a favorable prognosis. However, the authors reported the case of a primary ICC originating in the pineal gland presenting with recurrent thalamic hemorrhage and displaying an aggressive postoperative clinical course.
OBSERVATIONS
A 41-year-old man arrived at the emergency department with morning headaches and recurrent syncope that had lasted several months. Computed tomography and magnetic resonance imaging (MRI) revealed a pineal gland mass causing obstructive hydrocephalus and a subacute hematoma in the right thalamus. Three weeks after an endoscopic third ventriculostomy was performed, recurrent hemorrhage was observed in the right thalamus. The tumor was promptly removed surgically. The yellowish-white tumor did not exhibit abundant bleeding. No evidence of intratumoral hemorrhage around the hematoma pocket was found. Histopathological examination revealed the characteristics of a chordoma with minimal vascularity. MRI performed 10 weeks postoperatively for worsening headaches revealed abnormal enhancement of multiple cranial nerves, suggesting leptomeningeal seeding (LMS) of the tumor.
LESSONS
Despite radiotherapy and intrathecal chemotherapy, the patient’s neurological status worsened; he died 2 years postoperatively. A pineal ICC may cause recurrent thalamic hemorrhage and potentially fatal LMS, even in the early postoperative period.
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Affiliation(s)
- Ji-Eyon Kwon
- Departments of Neurosurgery and
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, South Korea
| | - So Young Ji
- Departments of Neurosurgery and
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, South Korea
| | - Kihwan Hwang
- Departments of Neurosurgery and
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, South Korea
| | - Kyu Sang Lee
- Pathology, Seoul National University Bundang Hospital, Gyeonggi-do, South Korea; and
| | - Gheeyoung Choe
- Pathology, Seoul National University Bundang Hospital, Gyeonggi-do, South Korea; and
| | - Chae-Yong Kim
- Departments of Neurosurgery and
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, South Korea
| | - Jung Ho Han
- Departments of Neurosurgery and
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, South Korea
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Sbardella E, Puliani G, Feola T, Pofi R, Pirchio R, Sesti F, Verdecchia F, Gianfrilli D, Moffat D, Isidori AM, Grossman AB. A clinical approach to parasellar lesions in the transition age. J Neuroendocrinol 2021; 33:e12995. [PMID: 34138496 DOI: 10.1111/jne.12995] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/19/2021] [Revised: 03/23/2021] [Accepted: 05/11/2021] [Indexed: 12/16/2022]
Abstract
Many reviews have summarised the pathology and management of the parasellar region in adult patients, although an analysis of these aspects in the transition years, from puberty onset to the age of peak bone mass, has been lacking. A comprehensive search of English-language original articles, published from 2000 to 2020, was conducted in the MEDLINE database (December 2019 to March 2020). We selected all studies regarding epidemiology, diagnosis and management of the following parasellar lesions: germinoma, craniopharyngioma, Langerhans cell histiocytosis, optic glioma, hypothalamic hamartoma, tuber cinereum hamartoma, cranial chordoma, Rathke cleft cyst, hypophysitis and hypothalamitis during the transition age from childhood to adulthood. In the present review, we provide an overview of the principal parasellar lesions occurring in the transition age. Symptoms are usually a result of the mass effect of the lesions on nearby structures, as well as anterior pituitary deficits. Diabetes insipidus occurs frequently in these patients. In this age group, pubertal developmental disorders may be more evident compared to other stages of life. Parasellar lesions in the transition age mostly include neoplastic lesions such as germinomas, hamartomas, optic gliomas, craniopharyngiomas Langerhans cell histiocytosis and chordomas, and rarely inflammatory lesions (hypophysitis, hypothalamitis). There are limited data on the management of parasellar lesions in the transition age. Endocrine evaluation is crucial for identifying conditions that require hormonal treatment so that they can be treated early to improve the quality of life of the individual patient in this complex age range. The clinical approach to parasellar lesions involves a multidisciplinary effort.
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Affiliation(s)
- Emilia Sbardella
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
| | - Giulia Puliani
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
- Oncological Endocrinology Unit, IRCCS Regina Elena National Cancer Institute, Rome, Italy
| | - Tiziana Feola
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
- Neuroendocrinology, Neuromed Institute, IRCCS, Pozzilli, Italy
| | - Riccardo Pofi
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
| | - Rosa Pirchio
- Dipartimento di Medicina Clinica e Chirurgia, Sezione di Endocrinologia, Università Federico II di Napoli, Naples, Italy
| | - Franz Sesti
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
| | - Federica Verdecchia
- Dipartimento Pediatrico Universitario Ospedaliero, Bambino Gesù Children Hospital, Rome, Italy
| | - Daniele Gianfrilli
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
| | - Daniel Moffat
- Department of Neurosurgery, Barts and the London NHS Trust, London, UK
| | - Andrea M Isidori
- Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy
| | - Ashley B Grossman
- Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, UK
- Centre for Endocrinology, Barts and the London School of Medicine, London, UK
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Abunimer A, Aiken A, Baugnon K, Wu X. Central Skull Base Anatomy and Pathology: A Review. Semin Ultrasound CT MR 2021; 42:266-280. [PMID: 34147162 DOI: 10.1053/j.sult.2021.04.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
The central skull base is an anatomically complex region of the head and neck which hosts a variety of neoplastic, vascular, infectious, inflammatory, and developmental pathologies. Evaluation of its intricate anatomy requires dedicated and complementary imaging modalities of MRI and CT. This article will provide a brief review of the anatomy of the central skull base, followed by an overview of common pathologies encountered in this region and their characteristic radiological characteristics.
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Affiliation(s)
- Ayman Abunimer
- Department of Radiology and Imaging Sciences, Division of Neuroradiology, Emory University School of Medicine, Atlanta, GA
| | - Ashley Aiken
- Department of Radiology and Imaging Sciences, Division of Neuroradiology, Emory University School of Medicine, Atlanta, GA
| | - Kristen Baugnon
- Department of Radiology and Imaging Sciences, Division of Neuroradiology, Emory University School of Medicine, Atlanta, GA
| | - Xin Wu
- Department of Radiology and Imaging Sciences, Division of Neuroradiology, Emory University School of Medicine, Atlanta, GA.
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D'Ortenzio R, Tolhurst S, Harvey M, Ghag R, Heran MK. The CT guided transoral approach: A biopsy technique for a poorly differentiated chordoma in a 5 year old. J Radiol Case Rep 2021; 15:1-8. [PMID: 34267865 DOI: 10.3941/jrcr.v15i3.4208] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
Mass lesions presenting at the craniocervical junction often present a unique challenge due to the complex anatomic arrangement limiting access for tissue diagnosis. The transoral approach has predominantly been used for percutaneous vertebroplasty of high cervical vertebrae with limited literature describing image guided biopsy for bony lesions in this region in the pediatric patient. We describe a technique of computed tomography guided transoral biopsy of a poorly differentiated chordoma located at the C1-C2 level in a 5-year-old child, and review this diagnosis.
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Affiliation(s)
- Robert D'Ortenzio
- Department of Radiology, Vancouver General Hospital, Vancouver, Canada
| | - Stefano Tolhurst
- Department of Radiology, Vancouver General Hospital, Vancouver, Canada
| | - Melissa Harvey
- Department of Pediatrics, BC Children's Hospital, Vancouver, Canada
| | - Ravi Ghag
- Department of Orthopaedics, BC Children's Hospital, Vancouver, Canada
| | - Manraj Ks Heran
- Department of Radiology, BC Children's Hospital, Vancouver, Canada
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