Barger James, Tanweer Omar, Liechty Benjamin, Snuderl Matija, Jafar Jafar J
Department of Neurosurgery, New York University School of Medicine, New York, USA.
Department of Pathology, New York University School of Medicine, New York, USA.
Surg Neurol Int. 2016 Sep 1;7(Suppl 23):S596-602. doi: 10.4103/2152-7806.189729. eCollection 2016.
Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin, which is clinically and histologically intermediate between benign hemangioma and angiosarcoma. It is most commonly found in the liver, lung, and bone, however, 46 intracranial cases have been reported in the literature, of which this is the fifth reported suprasellar tumor.
A 45-year-old woman developed progressive lethargy, somnolence, and memory decline over the course of 6 months. On computed tomography (CT), she was found to have a large hypothalamic mass and underwent subtotal resection via a bifrontal craniotomy.
While primary intracranial EHE is an uncommon presentation of a rare tumor, the suprasellar region does not seem to be an unusual location when it does occur. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere. Surgery is the first line of therapy, with variable benefit from adjuvant chemotherapy or radiation when total resection is not possible. Chemotherapeutic approaches in current use are directed at preventing endothelial proliferation.
上皮样血管内皮瘤（EHE）是一种罕见的血管源性肉瘤，在临床和组织学上介于良性血管瘤和血管肉瘤之间。它最常见于肝脏、肺和骨骼，然而，文献中已报道46例颅内病例，其中本例是第五例报道的鞍上肿瘤。
一名45岁女性在6个月内出现进行性嗜睡、昏睡和记忆力减退。计算机断层扫描（CT）显示她有一个巨大的下丘脑肿块，并通过双额开颅术进行了次全切除。
虽然原发性颅内EHE是一种罕见肿瘤的不常见表现，但当它发生时，鞍上区域似乎并非不寻常的位置。总体预后良好，原发性颅内疾病的预后可能比其他部位起源的EHE更好。手术是一线治疗方法，当无法进行全切除时，辅助化疗或放疗可能有不同程度的益处。目前使用的化疗方法旨在防止内皮细胞增殖。
