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Bialek S, Villeneuve LM, Bauer A, Phan M, Johnston A, Spence C, Graffeo CS. Neurosurgical Management of Spinal Epithelioid Hemangioendothelioma: Systematic Review and Illustrative Case Presentation. World Neurosurg 2024; 188:99-110. [PMID: 38734166 DOI: 10.1016/j.wneu.2024.05.006] [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: 02/09/2024] [Revised: 04/30/2024] [Accepted: 05/02/2024] [Indexed: 05/13/2024]
Abstract
BACKGROUND Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor predominantly arising in soft tissue. We report a rare case of thoracic spinal EHE with pulmonary metastasis. METHODS Case report and systematic review of spinal EHE. RESULTS A 36-year-old man presented with bilateral lower extremity weakness, progressive paresthesia, and urinary incontinence. He underwent open surgical resection of the tumor and decompression of the spinal cord, with subsequent improvement in neurologic function. Systematic review identified 84 cases of spinal EHE, 73 of which were primary, and 14 of which developed extra-spinal metastases. CONCLUSION EHE is an exceedingly rare tumor that may present with a wide swath of clinical symptoms. At present, no guidelines or formal treatment recommendations have been established. Surgical debulking has demonstrated efficacy as a front-line treatment, particularly in the setting of compressive neurologic dysfunction; data regarding adjuvant chemoradiation are less consistently reported, mandating further study.
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Affiliation(s)
- Shannan Bialek
- Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK
| | - Lance M Villeneuve
- Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK
| | - Andrew Bauer
- Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK
| | - Minh Phan
- Department of Internal Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK
| | - Andrea Johnston
- Department of Radiation Oncology, University of Oklahoma Health Sciences Center, Oklahoma City, OK
| | - Caple Spence
- Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK
| | - Christopher S Graffeo
- Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK.
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Villanueva-Castro E, Ramírez-Aragón S, Del Pino-Camposeco J, Canela-Calderon O, Ponce-Gómez JA, Arriada-Mendicoa JN. Spinal Cord Syndrome Due to Extramedullary Epithelioid Hemangioendothelioma of the Thoracic Spine: A Case Report and Literature Review. Cureus 2024; 16:e58571. [PMID: 38765371 PMCID: PMC11102575 DOI: 10.7759/cureus.58571] [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: 04/18/2024] [Indexed: 05/22/2024] Open
Abstract
We report a 48-year-old male patient with spinal epithelioid hemangioendothelioma in T3 and T4 who began with symptoms of paresthesia in the lower limbs and distal weakness of the right lower limb, back pain, increased limitation in walking, urinary incontinence, and constipation. A safe maximum resection was performed, finding residual disease during the PET/CT scan, so it was decided to treat with radiotherapy, and there was a good response to this treatment. A literature review of epithelioid hemangioendothelioma of the thoracic spine was done which showed a mean age of presentation of 41 years and a male-female ratio of 1:0.53. The main symptom was pain, which was present in 100% of the patients, and wide surgery was performed in 56.8% of the patients, intralesional surgery in 31.8%, and biopsy in 11.4%. A total of 46.6% of patients received radiation therapy, and only 6.6% received chemotherapy. The patients had an average follow-up of 38 months. We recommend that extension studies such as PET/CT scans be performed after surgical resection. This can serve as a follow-up with hemangioendothelioma epithelioma patients about metastatic disease or residual disease that will guide us in giving adjuvant treatments, such as radiotherapy or chemotherapy, for better control of the disease.
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Affiliation(s)
- Eliezer Villanueva-Castro
- Department of Neurosurgery, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX
| | | | - Jorge Del Pino-Camposeco
- Department of Neurosurgery, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX
| | - Obet Canela-Calderon
- Department of Neurosurgery, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX
| | - Juan Antonio Ponce-Gómez
- Department of Neurosurgery, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, MEX
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Tortorelli I, Bellan E, Chiusole B, Murtas F, Ruggieri P, Pala E, Cerchiaro M, Buzzaccarini MS, Scarzello G, Krengli M, Bisinella G, Battisti S, Di Maggio A, Zagonel V, Tos APD, Sbaraglia M, Brunello A. Primary vascular tumors of bone: A comprehensive literature review on classification, diagnosis and treatment. Crit Rev Oncol Hematol 2024; 195:104268. [PMID: 38237880 DOI: 10.1016/j.critrevonc.2024.104268] [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: 08/22/2023] [Revised: 12/23/2023] [Accepted: 01/11/2024] [Indexed: 02/02/2024] Open
Abstract
Primary vascular tumors of bone are a heterogeneous group of neoplasms, ranging from benign hemangiomas to frankly malignant epithelioid hemangioendotheliomas and angiosarcomas. Over the years, their classification has been a matter of discussion, due to morphologic similarities and uncertainty regarding biologic behavior. Over the past decade, with the development of next-generation sequencing, there has been a significant improvement in the molecular characterization of these lesions. The integration of their morphologic, immunohistochemical and molecular features has led to a better stratification, with important prognostic and therapeutic implications. Nevertheless, primary vascular bone tumors still represent a challenge for medical oncologists. Given their rarity and heterogeneity, in the last few years, there has been no significant progress in medical treatment options, so further research is needed. Here we present a review of the current knowledge regarding primary vascular tumors of the bone, correlating clinicopathologic features with tumor behavior and therapeutic approaches.
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Affiliation(s)
- Ilaria Tortorelli
- Oncology 1 Unit, Department of Oncology, Istituto Oncologico Veneto IOV - IRCCS, Via Gattamelata 64, 35128 Padua, Italy; Department of Surgery, Oncology and Gastroenterology (DISCOG), University of Padua, Via Nicolò Giustiniani 2, 35128 Padua, Italy
| | - Elena Bellan
- Department of Pathology, Azienda Ospedale Università Padova, Via Gabelli 61, 35121 Padua, Italy
| | - Benedetta Chiusole
- Oncology 1 Unit, Department of Oncology, Istituto Oncologico Veneto IOV - IRCCS, Via Gattamelata 64, 35128 Padua, Italy
| | - Fabio Murtas
- Oncology 1 Unit, Department of Oncology, Istituto Oncologico Veneto IOV - IRCCS, Via Gattamelata 64, 35128 Padua, Italy; Department of Surgery, Oncology and Gastroenterology (DISCOG), University of Padua, Via Nicolò Giustiniani 2, 35128 Padua, Italy
| | - Pietro Ruggieri
- Department of Orthopedics and Orthopedic Oncology, University of Padua, Via Nicolò Giustiniani 1, 35128 Padua, Italy
| | - Elisa Pala
- Department of Orthopedics and Orthopedic Oncology, University of Padua, Via Nicolò Giustiniani 1, 35128 Padua, Italy
| | - Mariachiara Cerchiaro
- Department of Orthopedics and Orthopedic Oncology, University of Padua, Via Nicolò Giustiniani 1, 35128 Padua, Italy
| | | | - Giovanni Scarzello
- Radiotherapy Unit, Istituto Oncologico Veneto IOV - IRCCS, Via Nicolò Giustiniani 2, 35128 Padua, Italy
| | - Marco Krengli
- Radiotherapy Unit, Istituto Oncologico Veneto IOV - IRCCS, Via Nicolò Giustiniani 2, 35128 Padua, Italy
| | - Gianluca Bisinella
- Division of Orthopedics and Trauma, AULSS 6 Euganea, Ospedali Riuniti Padova Sud, Via Albere 30, Monselice, Padua, Italy
| | - Sara Battisti
- Division of Orthopedics and Trauma, AULSS 6 Euganea, Ospedali Riuniti Padova Sud, Via Albere 30, Monselice, Padua, Italy
| | - Antonio Di Maggio
- Oncologic Radiology Unit, Department of Radiology and Medical Physics, Istituto Oncologico Veneto IOV - IRCCS, Via Gattamelata 64, 35128 Padua, Italy
| | - Vittorina Zagonel
- Oncology 1 Unit, Department of Oncology, Istituto Oncologico Veneto IOV - IRCCS, Via Gattamelata 64, 35128 Padua, Italy
| | - Angelo Paolo Dei Tos
- Department of Pathology, Azienda Ospedale Università Padova, Via Gabelli 61, 35121 Padua, Italy; Department of Medicine, University of Padua School of Medicine, Via 8 Febbraio 2, 35122 Padua, Italy
| | - Marta Sbaraglia
- Department of Pathology, Azienda Ospedale Università Padova, Via Gabelli 61, 35121 Padua, Italy; Department of Medicine, University of Padua School of Medicine, Via 8 Febbraio 2, 35122 Padua, Italy
| | - Antonella Brunello
- Oncology 1 Unit, Department of Oncology, Istituto Oncologico Veneto IOV - IRCCS, Via Gattamelata 64, 35128 Padua, Italy.
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Che F, Yang CW, Hu X, Li Q, Wei Y, Liu XJ, Song B. Massive Calcified Epithelioid Hemangioendothelioma With Multifocal Involvement: An Imaging Diagnosis Dilemma and a Rare Case Report. Front Oncol 2021; 11:782970. [PMID: 34976822 PMCID: PMC8718394 DOI: 10.3389/fonc.2021.782970] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2021] [Accepted: 11/30/2021] [Indexed: 02/05/2023] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor that develops from vascular endothelial or pre-endothelial cells. More than 60% patients have single-organ involvement, and involvement of multiple organs including the liver, lungs, and bones is extremely rare. The typical radiographic features of EHE include multiple small nodules in both lungs, which are usually located near small- and medium-sized blood vessels and the bronchi, and solitary, multiple, or diffuse lesions located at the hepatic periphery, spreading within the branches of the portal and hepatic veins. Radiologic calcification has been rarely reported in the literature. Here, we firstly described a case of a 53-year-old woman with EHE who presented with lungs, liver, bone, and right hilar lymph node involvement, manifesting as massive calcification on computed tomography. This case reminds physicians that EHE may present with unusual imaging manifestations, like massive calcification, and should be considered during the diagnostic process.
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Affiliation(s)
- Feng Che
- West China School of Medicine, West China Hospital, Sichuan University, Chengdu, China
| | - Cai wei Yang
- West China School of Medicine, West China Hospital, Sichuan University, Chengdu, China
| | - Xue Hu
- West China School of Medicine, West China Hospital, Sichuan University, Chengdu, China
| | - Qian Li
- West China School of Medicine, West China Hospital, Sichuan University, Chengdu, China
| | - Yi Wei
- West China School of Medicine, West China Hospital, Sichuan University, Chengdu, China
| | - Xi jiao Liu
- Department of Radiology, West China Hospital, Sichuan University, Chengdu, China
| | - Bin Song
- Department of Radiology, West China Hospital, Sichuan University, Chengdu, China
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A Review of the Spectrum of Imaging Manifestations of Epithelioid Hemangioendothelioma. AJR Am J Roentgenol 2020; 215:1290-1298. [PMID: 32841059 DOI: 10.2214/ajr.20.22876] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVE. The purpose of this article is to review the spectrum of imaging manifestations of epithelioid hemangioendothelioma across different organ systems and briefly describe its current treatment strategies. CONCLUSION. Epithelioid hemangioendothelioma is a rare, locally invasive neoplasm with metastatic potential. Although most commonly occurring in liver, lungs, and bones, it can also present at multiple other sites. Because of its nonspecific clinical and imaging manifestations, it is often misdiagnosed. The possibility of epithelioid hemangioendothelioma must be considered in the presence of a slowly growing mass that invades adjacent structures. Imaging can help plan percutaneous biopsy, detect sites of disease, and identify poor prognostic factors.
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Zhang XQ, Chen H, Song S, Qin Y, Cai LM, Zhang F. Effective combined therapy for pulmonary epithelioid hemangioendothelioma: A case report. World J Clin Cases 2020; 8:2009-2015. [PMID: 32518794 PMCID: PMC7262721 DOI: 10.12998/wjcc.v8.i10.2009] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/13/2020] [Revised: 04/13/2020] [Accepted: 04/24/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare disease. Thus far, consensus on a standard treatment for P-EHE has not been established given its low incidence worldwide. Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide has been used as an effective combination treatment for human malignancies. However, the efficacy of this combination has not been reported in P-EHE cases.
CASE SUMMARY We present the case of a 64-year-old woman with chest tightness, cough, and chest pain. Computed tomography showed multiple unresectable pulmonary nodules. She had been misdiagnosed with lung carcinoma and underwent gefitinib treatment at a hospital. Subsequently, the patient underwent a cardiothoracic surgery for further disease investigation. CD31, CD34, and Vimentin expression were detected in the resected nodule specimens by immunohistochemical analyses, and pathological analyses confirmed the diagnosis of P-EHE. Following this, four cycles of apatinib combined with chemotherapy with doxorubicin/cyclophosphamide were initiated. The patient demonstrated stabilization of multiple bilateral nodules and showed a dramatic improvement in the clinical presentation after combination treatment. The patient could not tolerate the side effects of chemotherapy. Therefore, she then continued apatinib monotherapy, which is ongoing to date. The patient was stable at the last follow-up after 24 mo.
CONCLUSION Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide may be an effective therapeutic option for P-EHE treatment.
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Affiliation(s)
- Xiu-Qin Zhang
- Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
| | - Heng Chen
- Department of Hematology Medicine, Wuxi People’s Hospital, Wuxi 214000, Jiangsu Province, China
| | - Shu Song
- Department of Pathology Medicine, Shanghai Public Health Clinical Center, Fudan University, Shanghai 200000, China
| | - Yan Qin
- Department of Pathology Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
| | - Li-Ming Cai
- Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
| | - Fang Zhang
- Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
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Challenges in Management of Epithelioid Hemangioendothelioma of the Spine: The Report of a Rare Cause of Cauda Equina Syndrome. ARCHIVES OF NEUROSCIENCE 2019. [DOI: 10.5812/ans.85206] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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Witte HM, Riecke A, Mayer T, Hackenbroch C, Steinestel K, Gebauer N, Puckhaber D, Mathieu R, Mauer UM, Schulz C. Multifocal and hormone-dependent epithelioid hemangioendothelioma with osteolysis of the second cervical vertebral body: report of an unprecedented surgical approach by using autologous bone graft. Br J Neurosurg 2019:1-9. [PMID: 31583911 DOI: 10.1080/02688697.2019.1668542] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Abstract
We report the case of a 28-year-old female patient who complained of extreme neck pain when giving birth to a child. Magnetic resonance imaging (MRI) of the cervical spine demonstrated an osteolytic lesion at the second cervical vertebral body (C2). In this presentation, we highlight a transoral surgical approach in order to prevent instability of this osteolytic lesion. To the best of our knowledge, this is the first time that such a route of access has been described for this tumor entity. A histopathologic examination led to the diagnosis of epithelioid hemangioendothelioma. During a follow-up period of 33 months, the patient had no complaints.
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Affiliation(s)
- Hanno M Witte
- Department of Hematology and Oncology, German Armed Forces Hospital of Ulm , Ulm , Germany.,Department of Hematology and Oncology, UKSH Campus Lübeck , Lübeck , Germany
| | - Armin Riecke
- Department of Hematology and Oncology, German Armed Forces Hospital of Ulm , Ulm , Germany
| | - Thomas Mayer
- Department of Hematology and Oncology, German Armed Forces Hospital of Ulm , Ulm , Germany
| | - Carsten Hackenbroch
- Department of Radiology, German Armed Forces Hospital of Ulm , Ulm , Germany
| | - Konrad Steinestel
- Institute of Pathology and Molecular Pathology, German Armed Forces Hospital of Ulm , Ulm , Germany
| | - Niklas Gebauer
- Department of Hematology and Oncology, UKSH Campus Lübeck , Lübeck , Germany
| | - Dirk Puckhaber
- Department of Neurosurgery, German Armed Forces Hospital of Ulm , Ulm , Germany
| | - René Mathieu
- Department of Neurosurgery, German Armed Forces Hospital of Ulm , Ulm , Germany
| | - Uwe Max Mauer
- Department of Neurosurgery, German Armed Forces Hospital of Ulm , Ulm , Germany
| | - Chris Schulz
- Department of Neurosurgery, German Armed Forces Hospital of Ulm , Ulm , Germany
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Bhatt AS, Muthusubramanian V, Kondappan A, Mahadevan A. Intradural Hemangioendothelioma of Lumbar Spine: Dilemmas and Differentials in Diagnosis. World Neurosurg 2019; 130:479-483. [PMID: 31295588 DOI: 10.1016/j.wneu.2019.06.172] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2019] [Revised: 06/21/2019] [Accepted: 06/22/2019] [Indexed: 10/26/2022]
Abstract
BACKGROUND Vascular tumors of the spine range from benign hemangiomas to malignant angiosarcomas. Hemangioendotheliomas of spine are tumors of intermediate-grade malignancy with rare occurrence in the intradural location. The imaging and histopathologic features may mimic other common lesions occurring at this location. CASE DESCRIPTION A 70-year-old woman presented with lower limb weakness and sensory impairment along the L5-S1 dermatome. A radiologic diagnosis of neurofibroma at the intradural location of the L4-L5 level was made, and total excision of the space-occupying lesion was done. Histopathologic examination, however, revealed a vascular tumor consistent with hemangioendothelioma, which was confirmed by immunohistochemical analysis. We present this case discussing the dilemmas and difficulties in arriving at the diagnosis, highlighting the role of immunohistochemical aid. CONCLUSION Histomorphology, aided by ancillar tests like immunohistochemistry, remains the criterion for the definitive diagnosis of such rare lesions, and pathologists need to be aware of these lesions occurring in uncommon locations.
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Affiliation(s)
- Anusha S Bhatt
- Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, India
| | - Vikram Muthusubramanian
- Department of Minimally Invasive Brain and Spine Surgery, Sri Ramakrishna Hospitals, Coimbatore, India
| | - Asokan Kondappan
- Department of Neurology, Sri Ramakrishna Hospitals, Coimbatore, India
| | - Anita Mahadevan
- Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
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Omerhodžić I, Bilalović N, Rovčanin B, Imširović B, Suljić E, Rotim A, Arnautović K. PRIMARY EPITHELIOID HEMANGIOENDOTHELIOMA IN THE CEREBELLUM: CASE REPORT WITH REFERENCE TO DRASTIC CHANGE IN THE WHO CLASSIFICATION. Acta Clin Croat 2018; 57:570-576. [PMID: 31168191 PMCID: PMC6536283 DOI: 10.20471/acc.2018.57.03.21] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/02/2023] Open
Abstract
SUMMARY – Epithelioid hemangioendothelioma is a rare vascular brain tumor. It develops from endothelial cells, usually in the liver, lung, bone and soft tissue. Primary localization of this tumor in the intracranial space is very uncommon; only 47 cases have been described in the literature. This tumor was initially classified as grade I (benign) in the World Health Organization (WHO) 2007 classification. In 2016, this tumor was re-classified as grade III (malignant). Herein, the first case report of epithelioid hemangioendothelioma in the cerebellum of a male patient is presented. Complete surgical excision was done. No adjuvant therapy was administered. Magnetic resonance imaging performed 2 years after the surgery continued to show no recurrence of the tumor. To our knowledge, this is the first report of cerebellar location of this rare tumor. In addition, the authors report drastic re-classification of the epithelioid hemangioendothelioma from the benign tumor (WHO 2007) to a malignant one (2016), which significantly changes postoperative management and follow up of this brain neoplasm.
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Affiliation(s)
| | - Nurija Bilalović
- 1Department of Neurosurgery, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 2Department of Clinical Pathology and Cytology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 3School of Medicine, University of Sarajevo, Sarajevo, Bosnia-Herzegovina; 4Department of Radiology, Sarajevo General Hospital, Sarajevo, Bosnia-Herzegovina; 5Department of Neurology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 6Department of Neurosurgery, Dubrava University Hospital, Zagreb, Croatia; 7Semmes-Murphey Clinic and University of Tennessee, Memphis, TN, USA
| | - Bekir Rovčanin
- 1Department of Neurosurgery, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 2Department of Clinical Pathology and Cytology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 3School of Medicine, University of Sarajevo, Sarajevo, Bosnia-Herzegovina; 4Department of Radiology, Sarajevo General Hospital, Sarajevo, Bosnia-Herzegovina; 5Department of Neurology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 6Department of Neurosurgery, Dubrava University Hospital, Zagreb, Croatia; 7Semmes-Murphey Clinic and University of Tennessee, Memphis, TN, USA
| | - Bilal Imširović
- 1Department of Neurosurgery, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 2Department of Clinical Pathology and Cytology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 3School of Medicine, University of Sarajevo, Sarajevo, Bosnia-Herzegovina; 4Department of Radiology, Sarajevo General Hospital, Sarajevo, Bosnia-Herzegovina; 5Department of Neurology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 6Department of Neurosurgery, Dubrava University Hospital, Zagreb, Croatia; 7Semmes-Murphey Clinic and University of Tennessee, Memphis, TN, USA
| | - Enra Suljić
- 1Department of Neurosurgery, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 2Department of Clinical Pathology and Cytology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 3School of Medicine, University of Sarajevo, Sarajevo, Bosnia-Herzegovina; 4Department of Radiology, Sarajevo General Hospital, Sarajevo, Bosnia-Herzegovina; 5Department of Neurology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 6Department of Neurosurgery, Dubrava University Hospital, Zagreb, Croatia; 7Semmes-Murphey Clinic and University of Tennessee, Memphis, TN, USA
| | - Ante Rotim
- 1Department of Neurosurgery, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 2Department of Clinical Pathology and Cytology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 3School of Medicine, University of Sarajevo, Sarajevo, Bosnia-Herzegovina; 4Department of Radiology, Sarajevo General Hospital, Sarajevo, Bosnia-Herzegovina; 5Department of Neurology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 6Department of Neurosurgery, Dubrava University Hospital, Zagreb, Croatia; 7Semmes-Murphey Clinic and University of Tennessee, Memphis, TN, USA
| | - Kenan Arnautović
- 1Department of Neurosurgery, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 2Department of Clinical Pathology and Cytology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 3School of Medicine, University of Sarajevo, Sarajevo, Bosnia-Herzegovina; 4Department of Radiology, Sarajevo General Hospital, Sarajevo, Bosnia-Herzegovina; 5Department of Neurology, University Clinical Center of Sarajevo, Sarajevo, Bosnia-Herzegovina; 6Department of Neurosurgery, Dubrava University Hospital, Zagreb, Croatia; 7Semmes-Murphey Clinic and University of Tennessee, Memphis, TN, USA
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Zheng Z, Wang H, Jiang H, Chen E, Zhang J, Xie X. Apatinib for the treatment of pulmonary epithelioid hemangioendothelioma: A case report and literature review. Medicine (Baltimore) 2017; 96:e8507. [PMID: 29137048 PMCID: PMC5690741 DOI: 10.1097/md.0000000000008507] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
Abstract
RATIONALE Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare tumor, with no established standard treatment. Overexpression of vascular endothelial growth factor receptor 2 (VEGFR-2) has been reported in some P-EHE patients. Apatinib, a new small molecule tyrosine kinase inhibitor that specifically targets VEGFR-2, has therapeutic benefits in some advanced tumors. However, its efficacy in P-EHE cases has not been reported. PATIENT CONCERNS Herein, we presented a 44-year-old man with recurrent hemoptysis for approximately 9 years. DIAGNOSES After hospitalization, relevant examinations were conducted. The disease was subsequently diagnosed as P-EHE. INTERVENTIONS The patient underwent pulmonary lobectomy, but subsequently developed multiple metastases. Within the tumor, CD31, CK, and Vimentin were found to be positive, while CD34 was negative. Apatinib was initially administered 250 mg daily doses and after 1 month was increased to 500 mg daily. OUTCOMES He showed noticeable symptomatic improvements and positive imaging changes in the first month of treatment. However, the disease progressed in the following month, despite the increased apatinib dose. LESSONS Apatinib is possibly a new treatment for P-EHE. However, further clinical trials are necessary to confirm an effective dose and the efficacy and safety of apatinib in P-EHE treatment.
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Affiliation(s)
| | | | - Hanliang Jiang
- Department of Respiratory Medicine, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, P.R. China
| | - Enguo Chen
- Department of Respiratory Medicine, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, P.R. China
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Ofluoglu AE, Aydogan M, Erdogan U, Ofluoglu O. Epithelioid hemangioendothelioma of the craniocervical junction; case report and review. ACTA ORTHOPAEDICA ET TRAUMATOLOGICA TURCICA 2017; 51:342-346. [PMID: 28478911 PMCID: PMC6197577 DOI: 10.1016/j.aott.2017.03.012] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/03/2015] [Revised: 03/27/2015] [Accepted: 12/24/2015] [Indexed: 11/26/2022]
Abstract
Epithelioid hemangioendotheliomas are uncommon vascular neoplasms and their spinal location is even rarer. We report clinical course of a 31-year-old man with an epithelioid hemangioendothelioma at the cranio-cervical junction. A cervical magnetic resonance imaging revealed tumor that caused posterior cervical cord compression. C1,2,3 total laminectomy and surgical excision of the tumor was performed. Postoperative external beam radiation was performed on the surgical field especially around the right vertebral artery. At 2-year follow-up there was no neurological deficit and no tumor recurrence.
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13
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Albakr A, Schell M, Drew B, Cenic A. Epithelioid hemangioendothelioma of the spine: case report and review of the literature. JOURNAL OF SPINE SURGERY 2017; 3:250-259. [PMID: 28744509 DOI: 10.21037/jss.2017.05.05] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Epithelioid hemangioendothelioma (EHE) has been described as a rare vascular bone lesion with histological features between hemangioma and high-grade angiosarcoma. Spinal EHE is a quite rare disease with few case reports and series reported in the literature. The tumor cells are positive for vimentin, CD31and CD34, factor VIII related antigen, ERG, and FLI1. Radiological features are not specific; it may appear as an osteolytic lesion. It can present as a multifocal disease in 40% of cases. No clear correlation with age and sex; however, it is slightly more common in males. Focal neck or back pain is the most common presenting symptom. The natural history of spinal EHE is unpredictable, and currently, there is no standard of care for treatment. Treatment options include preoperative embolization, and surgical resection followed by radiotherapy and/or chemotherapy. A 34-year-old previously healthy male presented with mid-thoracic back pain. Magnetic resonance imaging (MRI) of the spine revealed a decrease in vertebral body height at T5 with an enhancing mass. He underwent T5 balloon kyphoplasty and needle vertebral body biopsy. Results of the biopsy samples were non-diagnostic. Approximately 3 months after surgery, the patient presented with unsteady gait. A subsequent MRI revealed progression of the T5 compression fracture with cord compression. The patient subsequently underwent T4-T6 bilateral posterior decompression for epidural tumor and T3-T7 posterior instrumentation with pedicle screws. Pathology of the lesion revealed EHE. The patient was started on local radiation therapy (RT). On follow-up, 3 months after the second surgery, the thoracic spinal pain had improved dramatically. Our review highlights the diagnosis, clinical presentation, and treatment of spinal EHE. Complete resection is associated with good outcome. Radiotherapy has been used in partially resected lesions. However, the role of radiotherapy as primary treatment is not yet defined. Further studies should develop a treatment algorithm for this rare tumour.
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Affiliation(s)
- Abdulrahman Albakr
- Division of Neurosurgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
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14
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[Epithelioid hemangioendothelioma of the spine: A case report]. Cancer Radiother 2017; 21:222-225. [PMID: 28478891 DOI: 10.1016/j.canrad.2016.11.006] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2016] [Revised: 11/21/2016] [Accepted: 11/23/2016] [Indexed: 11/20/2022]
Abstract
with intensity modulation. During the follow-up, a second inoperable spinal location necessitated a new sequence of radiotherapy in modulation of intensity with integrated boost. The patient was alive at 24 months of follow-up without evidence of local relapse or distance. This observation is followed by a discussion with review of the literature on other epithelioid hemangioendothelioma cases treated with radiation.
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15
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Abstract
INTRODUCTION Epithelioid hemangioedothelioma (EHE) is a rare vascular tumor characterized by neoplastic proliferation of epithelioid or histiocytoid epithelial cells. EHE of bone constitutes <1% of primary malignant bone tumor. EHE in the skull is an extremely rare case. Here, we report a case of multiple neoplasm of the skull in a 15-month-old boy who presented with gradual facial swelling for 2 months. On computed tomography (CT) scan, multiple irregular osteolytic lesions were seen on the right maxillary, sphenoid, left zygoma, and roof of the left orbit. Excisional surgery of the lesion was planned. Histopathological and immunohistochemical examination of excised specimen suggest it to be epithelioid hemangioedothelioma. Follow-up for 6 months showed no recurrence. CONCLUSION Epithelioid hemangioedothelioma is a locally aggressive tumor with metastatic potential. CT imaging could help in assessment of lesion, but final diagnosis is possible only with histopathology. Complete surgical resection at the early stage of the disease is the most effective treatment with better prognosis.
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Affiliation(s)
| | - Miao Fan
- Department of Radiology
- Correspondence: Miao Fan, Department of Radiology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China. 510080 (e-mail: )
| | | | - Wei Liang
- Department of Medical Records Management, the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
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16
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Sebastian AS, Adair MJ, Morris JM, Khan MH, Arndt CAS, Nassr A. Minimally invasive treatment of a painful osteolytic lumbar lesion secondary to epithelioid hemangioendothelioma. Global Spine J 2015; 5:135-9. [PMID: 25844287 PMCID: PMC4369204 DOI: 10.1055/s-0034-1387198] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/25/2014] [Accepted: 06/02/2014] [Indexed: 01/25/2023] Open
Abstract
Study Design Case report. Objective Multifocal epithelioid hemangioendothelioma (EHE) of the spine is a rare disorder. We describe a novel, multimodal treatment of a painful osteolytic lumbar lesion secondary to EHE. The minimally invasive treatment results in an excellent patient outcome with decreased morbidity compared to traditional techniques. Methods A previously healthy young adult presented with a painful osteolytic lesion at the L2 vertebrae. Imaging revealed multifocal spinal lesions consistent with a history of EHE. Core needle biopsy confirmed the diagnosis. Preoperative cryoablation of L2 was followed by a staged surgery, which included a partial L2 corpectomy, tumor resection, bone grafting, and vertebral reconstruction using a minimally invasive technique. This treatment was followed by prolonged therapy with interferon and bisphosphonate. Results At 3.5 years' follow-up, the patient has maintained his vertebral body height, has not required a fusion, and has had no recurrence of disease. Conclusion Multimodal treatment consisting of tumor cryoablation, partial corpectomy, allograft reconstruction of the vertebrae, and adjuvant interferon and bisphosphonate can result in good outcomes for well-contained EHE tumors of the spine.
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Affiliation(s)
- Arjun S. Sebastian
- Department of Orthopedics, Mayo Clinic, Rochester, Minnesota, United States
| | - Marcus J. Adair
- Department of Orthopedics, Mayo Clinic, Rochester, Minnesota, United States
| | - Jonathan M. Morris
- Department of Radiology, Mayo Clinic, Rochester, Minnesota, United States
| | - Mustafa H. Khan
- Department of Orthopedics, Mayo Clinic, Rochester, Minnesota, United States
| | - Carola A. S. Arndt
- Department of Pediatrics, Mayo Clinic, Rochester, Minnesota, United States
| | - Ahmad Nassr
- Department of Orthopedics, Mayo Clinic, Rochester, Minnesota, United States,Address for correspondence Ahmad Nassr, MD Mayo Clinic, 200 First Street SWRochester, MN 55905United States
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17
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Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev 2014; 8:259. [PMID: 25992243 PMCID: PMC4419652 DOI: 10.4081/oncol.2014.259] [Citation(s) in RCA: 138] [Impact Index Per Article: 12.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2014] [Revised: 09/18/2014] [Accepted: 09/30/2014] [Indexed: 12/12/2022] Open
Abstract
Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.
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Affiliation(s)
- Angela Sardaro
- Department of Interdisciplinary Medicine, Section of Diagnostic Imaging and Radiotherapy, University Aldo Moro, Bari
| | - Lilia Bardoscia
- Department of Interdisciplinary Medicine, Section of Diagnostic Imaging and Radiotherapy, University Aldo Moro, Bari
| | - Maria Fonte Petruzzelli
- Department of Interdisciplinary Medicine, Section of Diagnostic Imaging and Radiotherapy, University Aldo Moro, Bari
| | - Maurizio Portaluri
- Department of Radiotherapy, A. Perrino Hospital, Brindisi
- Clinical Physiology Institute, National Research Council (IFC-CNR), Pisa-Lecce, Italy
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18
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Epithelioid hemangioendothelioma of the spine: results at seven years of average follow-up in a series of 10 cases surgically treated and a review of literature. EUROPEAN SPINE JOURNAL : OFFICIAL PUBLICATION OF THE EUROPEAN SPINE SOCIETY, THE EUROPEAN SPINAL DEFORMITY SOCIETY, AND THE EUROPEAN SECTION OF THE CERVICAL SPINE RESEARCH SOCIETY 2014; 24:2156-64. [PMID: 25118684 DOI: 10.1007/s00586-014-3510-9] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 05/05/2014] [Revised: 08/03/2014] [Accepted: 08/03/2014] [Indexed: 10/24/2022]
Abstract
PURPOSE To review a series of ten cases with epithelioid hemangioendothelioma of the spine, that have undergone surgery to describe clinical presentation, results and complications associated with surgical treatment; a review of literature reporting the main characteristics of the cases already published has been reported. METHODS A review of patients affected by epithelioid hemangioendothelioma surgically treated by the senior author from 1995 to 2012 was carried out. Ten cases were identified and clinical and radiological characteristics, therapy, complications and survival were valued. RESULTS Wide margin was achieved in two out of ten cases, marginal margin in seven and intralesional margin in one case. Average intraoperative blood loss was about 2,800 ml. Reported complications were one case of cord injury, one of dural tear, two cases of massive blood loss, a case of reconstruction failure, a wound dehiscence with deep infection, a pneumonia episode and a deep vein thrombosis with pulmonary embolism. Average follow-up was 84.4 months. Two local recurrences, after 32 and 37 months and two deaths for metastasis, after 14 and 36 months, were reported. Although several chemotherapy protocols are available for the treatment of EH of soft tissue, they are not relevant for the bone. CONCLUSIONS Wide surgery is probably associated with a better prognosis. Indeed most deaths and local recurrences reported in literature happened after intralesional surgery or chemotherapy/RT alone. The presenting study suggests that the best approach to achieve long-term local control and a major survival could be wide surgery, nevertheless more cases series are necessary to verify survival rate.
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19
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Sardaro A, Bardoscia L, Petruzzelli MF, Nikolaou A, Detti B, Angelelli G. Pulmonary epithelioid hemangioendothelioma presenting with vertebral metastases: a case report. J Med Case Rep 2014; 8:201. [PMID: 24942542 PMCID: PMC4070622 DOI: 10.1186/1752-1947-8-201] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2014] [Accepted: 04/07/2014] [Indexed: 01/02/2023] Open
Abstract
Introduction Epithelioid hemangioendothelioma is a rare vascular tumor that has an epithelioid and histiocytoid appearance, originates from vascular endothelial or pre-endothelial cells and comprises less than 1% of all vascular tumors. It was described for the first time in 1975 as pulmonary epithelioid hemangioendothelioma, because initially it was believed to be an aggressive form of bronchoalveolar cell carcinoma with a remarkable propensity to invade adjacent blood vessels and small airways. Only a few cases have been reported in the literature to date. Tumor cells expressing Fli-1 and CD31 have been identified as relatively specific endothelial markers. Epithelioid hemangioendothelioma may affect multiple organs and may vary considerably in its clinical and radiological presentation. More than 50% to 76% of pulmonary epithelioid hemangioendothelioma patients are asymptomatic. They are usually incidentally diagnosed on the basis of abnormal chest radiography during routine physical examinations. Hematologic and gastrointestinal disorders and weakness or numbness may also be observed, in addition to respiratory symptoms, in cases of disseminated pulmonary epithelioid hemangioendothelioma. Pain and swelling, pathological fractures, spine compression or paresthesia, loss of muscular strength and paraplegia may be present when bone metastases occur. Because of the rarity of this disease, there is no standard for treatment. Case presentation A 46-year-old Caucasian woman presented to our institution in November 2009 with metastases of pulmonary epithelioid hemangioendothelioma from the L3 and L4 vertebrae. A course of radiotherapy at a dosage of 3,000cGy delivered in individual doses of 200cGy/day for 5 days/wk to the L3 and L4 vertebrae led to the disappearance of the patient’s lumbar pain without any detectable side effects. Percussion of the patient’s vertebral spine was negative, and no radiological progression of bone disease was found at her 1-year follow-up examination. Conclusion Since epithelioid hemangioendothelioma was first correctly defined, several research groups have reported their experiences with epithelioid hemangioendothelioma irradiation. Further studies are needed to establish a standard radiation dose to be used for such a complex and extremely rare disease. In our present case, a radiotherapy dosage of 3,000cGy delivered in individual doses 200cGy/day for 5 days/wk allowed us to reach our goals: local pain control with good tolerance and better quality of life by the 1-year follow-up examination.
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Affiliation(s)
- Angela Sardaro
- Azienda Ospedaliero-Universitaria Policlinico di Bari, Dipartimento Interdisciplinare di Medicina, Sezione di Diagnostica per Immagini e Radioterapia, Università degli Studi di Bari "Aldo Moro", Piazza Giulio Cesare 11, 70124 Bari, Italia.
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20
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LANG JUNZHE, CHEN LEI, CHEN BI, CHEN KAI, LIU AIHI, LI JING, WANG JING. Epithelioid angiosarcoma of the spine: A case report of a rare bone tumor. Oncol Lett 2014; 7:2170-2174. [PMID: 24932310 PMCID: PMC4049740 DOI: 10.3892/ol.2014.2055] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/13/2013] [Accepted: 03/14/2014] [Indexed: 12/03/2022] Open
Abstract
Epithelioid angiosarcoma (EA) is an extremely rare subtype of angiosarcoma, which is characterized by large cells with an epithelioid morphology. EA typically arises in deep soft tissues, including the adrenal gland, skin and thyroid, however, EA rarely arises in the spine. The current study presents a case of osteolytic lesions involving the fourth lumbar (L4) level of the spine. Preoperatively, the patient was misdiagnosed with metastatic carcinoma, however, a radiological examination detected the presence of osteolytic or destructive lesions in the vertebrae, which extended into the pedicles. Histopathological and immunohistochemical evaluations were performed on the tumor tissue obtained from a decompression specimen of the L4 vertebra. A bone lesion composed of sheet-like malignant cells exhibiting atypical epithelioid morphology with vascular formation was observed. The presence of anastomosing vascular channels lined by epithelioid endothelial cells also indicated that focal endothelial differentiation had occurred. In addition, immunohistochemistry assays revealed that the lesion was positive for the endothelial cell markers, CD31, CD34 and vimentin. The tumor was treated with decompression of the L4 vertebra, followed by posterior stabilization. The patient subsequently refused chemotherapy and radiotherapy but completed six months of follow-up. At the time of writing, the tumor remains under control and the patient is asymptomatic. This case highlights the difficulty of diagnosing EA, which requires careful pathological examination and immunophenotype labeling. At present, CD31 is the most sensitive marker for detecting EA.
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Affiliation(s)
- JUNZHE LANG
- Department of Orthopedics, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China
| | - LEI CHEN
- Department of Orthopedics, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China
| | - BI CHEN
- Department of Orthopedics, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China
| | - KAI CHEN
- Department of Orthopedics, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China
| | - AIHI LIU
- Department of Gynecology, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China
| | - JING LI
- Department of Orthopedics, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China
| | - JING WANG
- Department of Orthopedics, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, P.R. China
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21
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Guy JB, Trone JC, Chargari C, Falk AT, Khodri M, Magné N. Epithelioid hemangioendothelioma of the spine treated with RapidArc volumetric-modulated radiotherapy. Med Dosim 2014; 39:242-5. [PMID: 24833300 DOI: 10.1016/j.meddos.2014.03.002] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2013] [Revised: 01/24/2014] [Accepted: 03/19/2014] [Indexed: 02/04/2023]
Abstract
Radiotherapy for epithelioid hemangioendothelioma (EHE) using volumetric intensity-modulated arc radiotherapy (VMAT). A 48-year-old woman was referred for curative irradiation of a vertebral EHE after failure of surgery. A comparison between VMAT and conventional conformal tridimensional (3D) dosimetry was performed and potential advantage of VMAT for sparing critical organs from irradiation's side effects was discussed. The total delivered dose on the planning target volume was 54 Gy in 27 fractions. The patient was finally treated with VMAT. The tolerance was excellent. There was no acute toxicity, including no increase in pain. With a follow-up of 18 months, no delayed toxicity was reported. The clinical response consisted of a decrease in the dorsal pain. The Dmax for the spinal cord was reduced from 55 Gy (3D-radiotherapy [RT]) (which would be an unacceptable dose to the spine because of the risk of myelopathy) to 42.8 Gy (VMAT), which remains below the recommended dose threshold (45 Gy). The dose delivered to 20% of organ volume (D20) was reduced from 47 Gy (3D-RT) to 3 Gy (VMAT) for the spinal cord. The study shows that VMAT allows the delivery of curative treatment for vertebral EHEs because of critical organ sparing.
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Affiliation(s)
- Jean-Baptiste Guy
- Department of Radiotherapy, Institut de Cancérologie de la Loire, St Priest en Jarez, France
| | - Jane-Chloé Trone
- Department of Radiotherapy, Institut de Cancérologie de la Loire, St Priest en Jarez, France
| | - Cyrus Chargari
- Department of Radiation Oncology, HIA du Val de Grâce, Paris, France
| | | | - Mustapha Khodri
- Department of Physics, Institut de Cancérologie de la Loire, St Priest en Jarez, France
| | - Nicolas Magné
- Department of Radiotherapy, Institut de Cancérologie de la Loire, St Priest en Jarez, France.
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22
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A multidisciplinary approach to the management of atypical osseous epithelioid hemangioendothelioma. Case Rep Oncol Med 2014; 2014:917425. [PMID: 24716052 PMCID: PMC3970260 DOI: 10.1155/2014/917425] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2013] [Accepted: 01/11/2014] [Indexed: 11/23/2022] Open
Abstract
Hemangioendothelioma is a rare vascular tumor of intermediate malignant potential. Though epithelioid hemangioendothelioma (EH) is commonly found in soft tissues, it has been known to be present in skeletal tissues. The authors present a case of a 50-year-old woman diagnosed with EH of the iliac bone and acetabulum, who experienced pathological fracture at presentation. This report describes a multidisciplinary approach to the management that includes initial incisional biopsy, curettage, and bone grafting, followed by Intensity Modulated Radiation Therapy. The patient finally underwent hemipelvic resection with allograft reconstruction after recurrence. Histopathological study revealed osseous EH of low mitotic activity that stained positively for CD31, CD34, vimentin, and Factor VIII. Herein, the authors discuss the imaging characteristics, histopathological aspects, cytogenetic findings, and the radiobiological behavior of osseous EH. After an aggressive multidisciplinary intervention, the patient is able to achieve local control with no evidence of distal metastatic disease.
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Fushimi K, Miyamoto K, Hioki A, Hosoe H, Takeuchi A, Shimizu K. Neurological deterioration due to missed thoracic spinal stenosis after decompressive lumbar surgery. Bone Joint J 2013; 95-B:1388-91. [DOI: 10.1302/0301-620x.95b10.31222] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
There have been a few reports of patients with a combination of lumbar and thoracic spinal stenosis. We describe six patients who suffered unexpected acute neurological deterioration at a mean of 7.8 days (6 to 10) after lumbar decompressive surgery. Five had progressive weakness and one had recurrent pain in the lower limbs. There was incomplete recovery following subsequent thoracic decompressive surgery. The neurological presentation can be confusing. Patients with compressive myelopathy due to lower thoracic lesions, especially epiconus lesions (T10 to T12/L1 disc level), present with similar symptoms to those with lumbar radiculopathy or cauda equina lesions. Despite the rarity of this condition we advise that patients who undergo lumbar decompressive surgery for stenosis should have sagittal whole spine MRI studies pre-operatively to exclude proximal neurological compression. Cite this article: Bone Joint J 2013;95-B:1388–91.
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Affiliation(s)
- K. Fushimi
- Kizawa Memorial Hospital, Department
of Orthopaedic Surgery, 590 Shimokobi, Kobichou, Minokamo
City, Gifu 505-8503, Japan
| | - K. Miyamoto
- Gifu University Graduate School of Medicine, Department
of Reconstructive Surgery for Spine, Bone and
Joints, 1-1 Yanagido, Gifu
City, Gifu 501-1194, Japan
| | - A. Hioki
- Gifu University Graduate School of Medicine, Department
of Orthopaedic Surgery, Gifu, Japan
| | - H. Hosoe
- Gifu Prefectural General Medical Centre, Department
of Orthopaedic Surgery, 4-6-1 Noishiki, Gifu
500-8717, Japan
| | - A. Takeuchi
- Kanazawa University Graduate School of
Medical Science, Department of Orthopaedic Surgery, 13-1
Takaramachi, Kanazawa 920-8640, Japan
| | - K. Shimizu
- Spine Centre, Gifu Municipal Hospital, Department
of Orthopaedic Surgery, 7-1 Kashima-chou, Gifu
500-8513, Japan
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24
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Munson TA, Valyi-Nagy T, Utset M, Lewis Z, Amin-Hanjani S. Intramedullary hobnail hemangioendothelioma of the conus. J Neurosurg Spine 2013; 18:515-8. [PMID: 23521687 DOI: 10.3171/2013.2.spine12730] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Hemangioendotheliomas have only rarely been encountered in the neuraxis. Here, the authors present a case of an intramedullary hobnail hemangioendothelioma of the spinal cord, the first case described of this particular pathological entity in the neuraxis. The authors discuss their treatment and review the pertinent literature regarding management.
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Affiliation(s)
- Troy A Munson
- Department of Neurosurgery, University of Illinois at Chicago, 912 South Wood Street, MC-799, Chicago, Illinois 60612, USA
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25
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Kerry G, Marx O, Kraus D, Vogel M, Kaiser A, Ruedinger C, Steiner HH. Multifocal epithelioid hemangioendothelioma derived from the spine region: case report and literature review. Case Rep Oncol 2012; 5:91-8. [PMID: 22539920 PMCID: PMC3337738 DOI: 10.1159/000336947] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022] Open
Abstract
Background Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with malignant biological behavior. It arises from endothelial cells, usually within soft tissues, and can occur in almost all locations. Case Report We report a unique case of a 25-year-old man who presented with sudden attacks of severe back pain followed by acute non-traumatic paraplegia. Emergency diagnostics revealed a pathologic fracture of the T7 vertebra with tumor tissue invasion of the spinal canal. Furthermore, multifocal metastases were found. Results To achieve en bloc resection, interdisciplinary surgical approaches were indicated. Despite multimodal therapy concepts, including radiotherapy and chemotherapy as well as endovascular embolization, the patient died within 8 weeks. Conclusion Prognosis of EHE is unpredictable and mainly determined by its location. The lesions are potentially aggressive; therefore, en bloc resection should be attempted whenever possible. However, as shown in the literature, only 15% of patients are suitable for total resection.
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Affiliation(s)
- G Kerry
- Department of Neurosurgery, Klinikum Nuernberg, Nuremberg, Germany
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26
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Ma J, Wang L, Mo W, Yang X, Xiao J. Epithelioid hemangioendotheliomas of the spine: clinical characters with middle and long-term follow-up under surgical treatments. EUROPEAN SPINE JOURNAL : OFFICIAL PUBLICATION OF THE EUROPEAN SPINE SOCIETY, THE EUROPEAN SPINAL DEFORMITY SOCIETY, AND THE EUROPEAN SECTION OF THE CERVICAL SPINE RESEARCH SOCIETY 2011; 20:1371-6. [PMID: 21512841 DOI: 10.1007/s00586-011-1798-2] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/11/2009] [Revised: 03/03/2011] [Accepted: 04/08/2011] [Indexed: 01/04/2023]
Abstract
Epithelioid hemangioendothelioma, an aggressive vascular tumor has the rarity of morbidity that arises in the spine. There were few cases reported in literatures in recent years, and little was known about this disease. A review study of the patient files in our constitutions between 1996 and 2006 showed that five patients were treated for spinal epithelioid hemangioendothelioma. Although only five patients, this study attempts to bring more informations about this rare lesion. This patient group included two males and three females. The lesions located in the cervical (case 1) or thoracic (case 2-4) or lumbar spine (case 5). Treatments included: laminectomy and cytoreductive surgery followed by external beam irradiation (one patient), expanded resection in piece meal with postoperative external beam irradiation (three patients), and total en bloc resection alone (one patient). Reconstruction of the spinal stability was performed in four patients. Follow-up period ranged from 25 to 72 months, averaged 47.4 months. The neurologic function of patients got a satisfactory progress except the paraplegic patient at diagnosis. The patient who received laminectomy and cytoreductive surgery followed by external beam irradiation still presented with tumor local progress, metastasis, and she died at 34 months after operation. No local recurrence or distant metastasis was detected in the other four patients. Epithelioid hemangioendothelioma of the spine is so rare in clinic as a primary aggressive vascular tumor. Based on our experience, a valid expanded resection of the tumor with adjunct radiation therapy or total en bloc excision may present with acceptable results.
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Affiliation(s)
- Junming Ma
- Department of Orthopaedics, LongHua Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China
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Complicated surgical resection of malignant tumors in the upper cervical spine after failed ion-beam radiation therapy. Spine (Phila Pa 1976) 2010; 35:E505-9. [PMID: 20421861 DOI: 10.1097/brs.0b013e3181caa86c] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Abstract
STUDY DESIGN Case report. OBJECTIVE To report 3 cases of malignant tumors in the upper cervical spine that were treated surgically by a combination of posterior tumor resection and stabilization and anterior tumor resection through a mandible-splitting approach after failed ion-beam radiation therapy. SUMMARY OF BACKGROUND DATA Few clinical reports have described in detail the postoperative complications associated with transoral surgical resection of tumors in the upper cervical spine after unsuccessful ion-beam radiation therapy. METHODS Three patients with malignant tumors in the upper cervical spine who had undergone ion-beam radiotherapy and experienced tumor recurrence were treated by a combination of posterior and anterior surgery through a mandible-splitting approach. One patient (patient 1, a 32-year-old man) had a hemangioendothelioma at the C2 and C3 level, whereas the other 2 patients (patient 2, a 66-year-old woman and patient 3, a 65-year-old man) had a chordoma at the C2 and C3 level. RESULTS The intralesional but macroscopic total resection of the tumors was achieved in all 3 patients. However, serious complications developed after surgery, including deep wound infection, cerebrospinal fluid leakage, and meningitis in patient 1, prolonged swallowing difficulty, subsidence of the strut graft, and recurrence in patient 2, and deep wound infection and discitis causing progressive paralysis in patient 3. All patients underwent salvage surgery, including debridement of the wound in patient 1, posterior reinforcement using instrumentation and posterior tumor resection for the recurrent tumor in patient 2, and anterior debridement of the wound with a pedicle flap using the pectoral major muscle in patient 3 to address these problems. Patients 1 and 3 had no signs of recurrence at the time of a follow-up examination. CONCLUSION Severe complications, mainly associated with the disturbance in healing of the retropharyngeal wall, were observed in all 3 patients. A preventive method, such as primary repair of the retropharyngeal wall using muscular/musculocutaneous flaps, should be considered for patients undergoing resection through a transoral approach after ion-beam irradiation.
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Kopniczky Z, Tsimpas A, Lawson DDA, King A, Pohl U, Pollock JR, David KM. Epithelioid hemangioendothelioma of the spine: report of two cases and review of the literature. Br J Neurosurg 2009; 22:793-7. [PMID: 19085365 DOI: 10.1080/02688690802158694] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Abstract
We report two patients with spinal epithelioid hemangioendothelioma (EH), an uncommon vascular, potentially malignant tumour. Neurological signs, diagnostic images, surgical techniques and complications, histology and the role of adjuvant therapy are discussed. Primary manifestation of EH of the vertebral column is rare. Thorough preoperative clinical and radiological workup, radical surgical excision, and close postoperative follow-up are recommended.
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Affiliation(s)
- Z Kopniczky
- Department of Neurosurgery, Essex Centre for Neurosciences, Queen's Hospital, Romford, UK
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