Abstract
Intracranial epithelioid hemangioendothelioma (E-HE) is an extremely rare, vasoformative tumor with variable biological behavior. A huge E-HE confined to the upper side of the tentorium has not been documented previously. Recognizing E-HE as a specific vascular tumor is important, and complete tumor removal guarantees long-term survival. Here, we report an intracranial E-HE of a 24-year-old woman with the initial symptoms of progressive headache over several years. Magnetic resonance imaging revealed a huge tumor attached to the upper side of the right tentorium, with tentorial and tonsillar herniation, and secondary syringomyelia in the upper cervical spinal cord. The initial surgical attempt to remove the lesion was abandoned due to profuse bleeding from the tumor. One year after irradiation and 5 days after vascular embolization, the tumor could be removed totally. The final definitive diagnosis as E-HE was made by special immunohistochemical and electron microscopic study. The patient is free from the tumor 9 years after the second operation. Thus, E-HE is a highly vascular-rich tumor to excise, and we stress need for preoperative embolization and/or irradiation to remove huge tumor completely.
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Enzinger FM, Weiss SW: Hemangioendothelioma, in Soft Tissue Tumors. 3rd edn., St. Lous, Mosby, 1995, pp 891-915
Weiss SW, Enzinger FM: Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50: 970–981, 1982.
Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM: Epithelioid hemangioendothelioma and related lesions. Semin Diag Pathol 3: 259–287, 1986
Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, Patterson SD, Hammar SP: Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT): an analysis of twenty cases of a peculiar sclerosing endothelial tumor. Cancer 51: 452–464, 1983
Ishak KG, Sesterhenn IA, Goodman MZD: Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol 15: 839–852, 1984
Kleer CG, Unni KK, Mcleod RA: Epithelioid hemangioendothelioma of bone. Am J Surg Pathol 20: 1301–1311, 1996
Ellis GL, Kratochvil III FJ: Epithelioid hemangioendothelioma of the head and neck: a clinicopathologic report of twelve cases. Oral Surg Oral Med Oral Pathol 61: 61–68, 1986
Chen TC, Gonzalez-Gomez I, Gilles FH, Mccomb JG: Pediatric intracranial hemangioendotheliomas: case report. Neurosurgery 40: 410–414, 1997
Chow LT, Chow W, Fong DT: Epithelioid hemangioendothelioma of the brain. Am J Surg Pathol 16: 619–625, 1992
Fryer JA, Biggs MT, Katz IA, Brazier DH, Shakespeare TP: Intracranial epithelioid hemangioendothelioma arising at site of previously excised atypical meningioma. Pathology 30: 95–99, 1998
Hodaie M, Becker L, Teshima I, Rutka JT: Total resection of an intracerebral hemangoendothelioma in an infant. Pediatr Neurosurg 34: 104–112, 2001
Hurley TR, Whisler WW, Clasen RA, Smith MC, Bleck TP, Doolas A, Dampier MF: Recurrent Intracranial epithelioid hemangioendothelioma associated with multicentric disease of liver and heart: case report. Neurosurgery 35: 148–151, 1994
Kepes JJ, Rubinstein LJ, Maw G, Burdick: Epithelioid hemangiomas (hemangioendotheliomas) of the central nervous system and its coverings, a report of three cases. J Neuropathol Exp Neurol 45: 319, 1986
Llena JF, Hirano A, Inoue A: Vasoformative tumor of the brain-immunohistology and ultrastructure. Clin Neuropathol 3: 155–159, 1984
Matturri L, Ottaviani G, Rossi L: Sudden and unexpected infant death due to an hemangioendothelioma located in the medulla oblongata. Adv Clin Path 3: 29–33, 1999
Nora FE, Scheithauer BW: Primary epithelioid hemangioendothelioma of the brain. Am J Surg Pathol 20: 707–714, 1996
Ortega-Aznar A, Vidal FR, Mendez EV, Grassa BI: Intracranial hemangioendotheliomas. Int J Neuroradiol 4: 366–372, 1998
Palmieri G, Montella L, Martignetti A, Bianco AR: Interferon α-2b at low doses as long-term antiangiogenic treatment of a metastatic intracranial hemangioendothelioma: a case report. Oncol Rep 7: 145–149, 2000
Pearl GS, Takei Y, Tindall GT, O'Brien MS, Payne NS, Hoffman JC: Benign hemangioendothelioma involving the central nervous system: 'strawberry nevus' of the neuraxis. Neurosurgery 7: 249–256, 1980
Phookan G, Davis AT, Holmes B: Hemangioendothelioma of the cavernous sinus: case report. Neurosurgery 42: 1153–1156, 1998
Puca A, Meglio M, Rollo M, Zannoni GF: Intracranial epithelioid hemangioendothelioma: case report. Neurosurgery 38: 399–401, 1996
Tammam AG, Lewis PD, Crockard HA: Cerebello-pontine angle epithelioid hemangioendothelioma in a 4-year-old boy. Child's Nerv Syst 13: 648–650, 1997
Tancredi A, Puca A, Carbone A: Multifocal cerebral hemangioendothelioma. Case report and review of the literature. Acta Neurochir 142: 1157–1161, 2000
Taratuto AL, Sevlever ZG, Saccoliti SM: Epithelioid hemangioendothelioma of the central nervous system. Pediatr Neurosci 14: 11–14, 1988.
Golash A, Strang FA, Reid H: Intracranial haemangioendothelioma mimicking a meningioma. Br J Neurosurg 13: 594–597, 1999
Avellino AM, Grant GA, Harris AB, Wallace SK, Shaw CM: Recurrent intracranial Masson's vegetant intravascular hemangioendothelioma. J Neurosurg 91: 308–312, 1999
Miettinen M, Lindenmayer AE, Chaubal A: Endothelial cell markers CD31, CD34, and BNH9 antibody to H-and Y-antigens — evaluation of their specificity and sensitivity in the diagnosis of vascular tumors and comparison with von Willebrand factor. Mod Pathol 7: 82–90, 1994
Pearl GS, Takei Y: Hemangioendothelioma of the neuraxis: an ultrastructural study. Neurosurgery 11: 486–490, 1982
Mena H, Ribas JL, Enzinger FM, Parisi JE: Primary angiosarcoma of the central nervous system: study of eight cases and review of the literature. J Neurosurg 75: 73–76, 1991
Samaratunga H, Searle J, Cominos D, Fevre IL: Cerebral metastasis of an atrial myxoma mimicking an epithelioid hemangioendothelioma. Am J Surg Pathol 18: 107–111, 1994
Kleihues P, Cavenee WK (eds): WHO Classification of Tumours of the Nervous System, in Pathology & Genetics, Tumours of the Nervous System, World Health Organization Classification of Tumours, IARC Press, Lyon, 2000, pp 6-7
Fukui M, Kitamura K, Nakagaki H, Yamakawa Y, Kinoshita K, Hayabuchi N, Jingu K, Numaguchi Y, Matsuura K, Watanabe K: Irradiated meningiomas: a clinical evaluation. Acta Neurochir 54: 33–43, 1980
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Kubota, T., Sato, K., Takeuchi, H. et al. Successful Removal After Radiotherapy and Vascular Embolization in a Huge Tentorial Epithelioid Hemangioendothelioma: A Case Report. J Neurooncol 68, 177–183 (2004). https://doi.org/10.1023/B:NEON.0000027760.84461.c7
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DOI: https://doi.org/10.1023/B:NEON.0000027760.84461.c7