血管外皮细胞瘤及其在中枢神经系统的表现

目的分析血管外皮细胞瘤(HPC)在各系统特别是在中枢神经系统的临床表现及其治疗。方法结合有关文献，回顾解放军总医院近10年内通过手术治疗的41例HPC，并对不同系统发生的HPC进行比较。结果41例HPC中，中枢神经系统21例(51．2％)，其他系统20例(48．8％)，13例诊断为恶性HPC。41例HPC患者共接受手术62次，肿瘤全切49次，次全切13次。其中14例患者总计复发21次(33．9％)。转移5例(12．1％)，死亡5例。中枢神经系统的HPC与其他系统的HPC临床表现相似，病理形态相同。结论HPC预后不良，复发率和转移率很高，手术全切结合放疗有一定疗效。中枢神经系统的HPC应和其他系统的HPC归为一种疾病来认识，但也需注意其自身特点。

Hemangiopericytoma and its manifestations in central nervous system(CNS)

Objective To elucidate the clinical features and treatment of hemangiopericytomas (HPCs) in different parts of the body, especially in CNS. Methods Clinical data of 41 cases with HPC which were treated at 301 Hospital from 1993 to 2004 were retrospectively analyzed with a review of relevant literature. Follow-up periods ranged from 4 to 150 months after surgery (mean 34 months). Results Among 41 patients of HPC, 21 tumors (51.2%) were found in CNS, and the rest 20 (48.8%) in other systems. Thirteen tumors were diagnosed as malignant HPC. Surgery for tumor removal was performed in all 41 patients. Among the 62 operations, total removal of tumor was achieved in 49 cases and subtotal removal in 13 cases. Recurrence in original site of the tumor occurred 21 times (33.9%) in 14 patients. Five patients (12.1%) presented one or more distant metastasis, and 5 patients died. HPCs in CNS and in other systems displayed similar clinical characteristics and pathological morphology. Conclusions HPC is a rare angioneoplasm that may arise in any part of the body. It is a great challenge to neurosurgeons as well as other surgeons to surgically manage the tumor. HPC has an unfavorable prognosis because its high rate of recurrence and metastasis. HPC in CNS and in other parts of the body should be recognized as one disease. At the same time, HPC in CNS has its distinct features.