Batista Kelvin Piña, Gómez Gonzalo Lepe, Quintana Eduardo Murias, Astudillo Aurora, Fernandez-Vega Ivan, Fernandez Belen Alvarez, Cuellar-Martínez Ana, Llorente-Pendás José, Rovira-Pereira Veronica, Alvarez-Reyes Kenia
Hospital Universitario Central de Asturias, Spain.
Hospital Clínico Universitario de Valladolid, Spain.
Contemp Oncol (Pozn). 2018;22(2):118-123. doi: 10.5114/wo.2018.76235. Epub 2018 Jun 13.
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.
上皮样血管内皮瘤（EHE）是一种罕见的低级别血管肿瘤，主要由上皮样细胞组成。EHE可表现为孤立性肿瘤或多发性全身病变，常见于软组织、肝脏、胸膜、肺、腹膜、淋巴结、乳腺及许多其他部位。颅鼻区域的EHE极为罕见。关于颅底EHE的病例报道非常少。我们讨论了一例起源于鞍区的侵袭性EHE的极其罕见的病例。基于文献回顾，我们的患者是首例报道的巨大孤立性EHE，侵犯脑桥前池并压迫展神经，酷似软骨肉瘤。考虑到在海绵窦和斜坡区域实现肿瘤完整切除且切缘阴性，同时避免侵犯神经和血管结构在技术上具有难度，我们对这例罕见肿瘤进行了近全手术切除并随后进行放疗。
