eISSN: 1897-4309
ISSN: 1428-2526
Contemporary Oncology/Współczesna Onkologia
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vol. 22
Case report

Giant cranionasal epithelioid haemangioendothelioma with invasive growth pattern mimicking a skull base chondrosarcoma

Kelvin Piña Batista, Gonzalo Lepe Gómez, Eduardo Murias Quintana, Aurora Astudillo, Ivan Fernandez-Vega, Belen Alvarez Fernandez, Ana Cuellar-Martínez, José Llorente-Pendás, Veronica Rovira-Pereira, Kenia Alvarez-Reyes

Contemp Oncol (Pozn) 2018; 22 (2): 118-123
Online publish date: 2018/06/13
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Epithelioid haemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that is composed of mostly epithelioid cells. EHE may arise as a solitary tumour or in the form of multiple body lesions, and commonly occurs in soft tissues, liver, pleura, lung, peritoneum, lymph nodes, breast, and many other sites. EHE in the cranionasal region is extremely rare. There are very few reports of cases of skull-base EHE. We discuss an extremely rare presentation of an aggressive EHE that originated from the sellar region. Based on literature review, our patient is the first reported case of a giant solitary EHE with prepontine cistern invasion and abducens nerve encroachment mimicking a chondrosarcoma. We treated this rare tumour by near subtotal surgical excision with subsequent radiotherapy, considering that complete tumour resection with free margins in both cavernous sinus and clival region avoiding neural and vascular structure encroachment becomes technically difficult.

epithelioid haemangioendothelioma, vascular tumour, skull base, cranionasal

Fernandes AL, Ratilal B, Mafra M, Magalhaes C. Aggressive intracranial and extra-cranial epithelioid hemangioendothelioma: A case report and review of the literature. Neuropathology 2006; 26: 201-205.
Aniba K, Laghmari M, Lmejjati M, Ghannane H, Ait Benali S. A Tragical Paediatric Case History of Intraorbital and Intracranial Epithelioid Hemangioendothelioma. Case Rep Neurol Med 2012; 396097.
Naqvi J, Ordonez NG, Luna MA, Williams MD, Weber RS, El-Naggar AK. Epithelioid Hemangioendothelioma of the Head and Neck: Role of podoplanin in the differential diagnosis. Head Neck Pathol 2007; 2: 25-30.
Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev 2014; 8: 1-10.
Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 1982; 50: 970-981.
Adler B, Naheedy J, Yeager N, Nicol K, Klamar J. Multifocal epithelioid hemangioendothelioma in a 16-year-old boy. Pediatr Radiol 2005; 35: 1014-1018.
Medina M, Polo R, Vaca M, Alonso A, Cobeta I. Multifocal epithelioid hemangioendothelioma with massive lateral skull base involvement. Otol Neurotol 2015; 36: e67-69.
Ma SR, Li KC, Xu YQ, Wang YM, Ma WL, Li Q. Primary epithelioid hemangioendothelioma in the clival region: A case report and literature review. Neuropathology 2011; 31: 519-522.
Rushing EJ, White JA, D’Alise MD, Chason DP, White CL 3rd, Bigio EH. Primary epithelioid hemangioendothelioma. Clin Neuropathol 1998; 17: 110-114.
Watanabe T, Saito N, Shimaguchi H, Fujimaki H, Kamiya M, Nakazato Y, Sasaki T. Primary epithelioid hemangioendothelioma originating in the lower petroclival region: case report. Surg Neurol 2003; 59: 429-433; discussion 434.
Ogita S, Endo T, Nomura K, et al. Nasal cavity epithelioid hemangioendothelioma invading the anterior skull base. Surg Neurol Int 2016; 7: 53-57.
Patnayak R, Jena A, Reddy K, Chowhan A, Rao CM, Rukhamangadha N. Epithelioid hemangioendothelioma of nasal cavity. J Lab Physicians 2010; 2: 111-115.
Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F (Eds). World Health Organization Classification of Tumours. Pathology and genetics of tumours of soft tissue and bone. IARC Press: Lyon 2013.
Miettinen M, Wang Z, Lasota J, Heery C, Schlom J, Palena C. Nuclear Brachyury Expression Is Consistent in Chordoma, Common in Germ Cell Tumors and Small Cell Carcinomas, and Rare in Other Carcinomas and Sarcomas. Am J Surg Pathol 2015; 39: 1305-1312.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P. The 2007 WHO Classification of Tumours of the Central Nervous System. Acta Neuropathol 2007; 114: 97-109.
Wang L, Wu Z, Tian K, Li G, Zhang J. Clinical and Pathological Features of Intradural Retroclival Chordoma. World Neurosurg 2014; 82: 791-798.
Piña Batista KM, Álvarez KY, Lopez FP, et al. Immunophenotypic features of dedifferentiated skull base chordoma: an insight into the intratumoral heterogeneity. Contemp Oncol 2017; 21: 267-273.
Sekhar LN, Pranatartiharan R, Chanda A, Wright DC. Chordomas and chondrosarcomas of the skull base: results and complications of surgical management. Neurosurg Focus 2001; 10: E2.
Hornick JL (Ed.). Practical Soft Tissue Pathology: A Diagnostic Approach. Elsevier/Saunders: Philadelphia 2013.
Barresi V, Ieni A, Branca G, Tuccari G. Review Article Brachyury: A Diagnostic Marker for the Differential Diagnosis of Chordoma and Hemangioblastoma versus Neoplastic Histological Mimickers. Dis Markers 2014; 514753.
Zhu Y, Fan M, Pandey S, Liang W, Chang D. Multiple epithelioid hemangioedothelioma of the skull in a child. Medicine (Baltimore) 2016; 95: e4081-4083.
Pamir MN, Ozduman K. Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas. Eur J Radiol 2006; 58: 461-470.
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