Parajón Avelino, Vaquero Jesús
Department of Neurosurgery, Puerta de Hierro Hospital, Autonomous University, San Martín de Porres, Madrid, Spain.
J Neurooncol. 2008 Jun;88(2):169-73. doi: 10.1007/s11060-008-9543-3.
Intracranial epithelioid hemangioendothelioma (EH) is a very rare vascular neoplasm. We describe a case of meningeal intracranial tumor in a 58 year old man, located in the greater wing of the sphenoid bone. The tumor was completely excised via pterional approach and the pahological analysis showed a hemorrhagic lesion with capillary-sized vessels lined by atypical-appearing endothelial cells that expressed CD31, CD34 and Factor VIII antigens. A diagnosis of EH was established, and one year after surgery the patient is symptom-free, without tumor recurrence or residual tumor. A review of the literature disclosed other 34 cases of intracranial EH. In these tumors, total resection is mandatory if possible, otherwise radiotherapy is recommended. Outcome is favorable in the majority of cases.
颅内上皮样血管内皮瘤(EH)是一种非常罕见的血管性肿瘤。我们描述了一例58岁男性的脑膜颅内肿瘤,位于蝶骨大翼。通过翼点入路将肿瘤完全切除,病理分析显示为一个出血性病变,有由非典型内皮细胞衬里的毛细血管大小的血管,这些内皮细胞表达CD31、CD34和因子VIII抗原。确诊为EH,术后一年患者无症状,无肿瘤复发或残留肿瘤。文献回顾发现另外34例颅内EH病例。对于这些肿瘤,若可能应尽量全切,否则建议放疗。大多数病例预后良好。