腰椎软组织上皮样血管内皮瘤临床病理观察

Epithelioid hemangioendothelioma involving soft parts of the lumbar vertebra: a clinicopathological analysis

目的探讨上皮样血管内皮瘤(EHE)的临床病理特点及鉴别诊断。方法观察l例腰椎软组织EHE的病理组织学表现及免疫组化染色,同时复习相关文献。结果患者女性,29岁。4年前无明显诱因出现腰痛,伴有下肢放射痛。MRI示腰4、5椎体水平腹膜后、椎旁异常信号肿块,累及椎体及邻近结构。手术切除肿物及椎体。镜下肿瘤细胞呈上皮样,胞质嗜酸,可见胞质内空泡,内含红细胞。免疫组化:肿瘤细胞vimentin、CD31和laminin(+),CD34、CD68、FⅧRAg、CK、S-100、SMA、Syn和CD56(-),Ki-67阳性指数为10%。随访16个月未复发或转移。结论 EHE是一种少见的血管肿瘤,易误诊为转移癌或肌上皮肿瘤,依据其独特的病理组织学特点及免疫组化可做出正确诊断。

Objective To investigate the clinicopathological features and differential diagnosis of epithelioid hemangioendothelioma（ EHE）. Methods We observed one case of EHE involving soft parts of the lumbar vertebra,using histopathological and immunohistochemical staining,with review of the related literatures. Results A 29-year-old woman presented with lumbodynia and radiating pain in the lower extremities without reason.Magnetic resonance imaging（ MRI） revealed of lumbar paravertebra 4 and 5 and retroperitonal abnormal signal intensity masses,involving the vertebra and the surrounding tissues. So the tumor and the vertebra were resected.Histopathological features of the tumor cells were epithelioid,with eosinophilic cytoplasm and cytoplasmic vacuoles which occasionally contained red blood cells. Immunohistochemical staining showed the tumor cells positive for vimentin,CD31,laminin,and negative for CD34,CD68,F Ⅷ RAg,CK,S-100,SMA,Syn and CD56; Ki-67 labeling index was 10 %. No tumor recurrence or metastasis was found by the following-up for one year and four months. Conclusions EHE is a rare vascular tumor,easily misdiagnosed as metastatic carcinoma or myoepithelial tumor. A proper diagnosis would be established according to its unique histopathological characteristics and immunohistochemical staining.