临床与实验病理学杂志2012，Vol.28Issue(9)：1006-1011,6.
原发性中枢神经系统平滑肌肉瘤的临床病理分析
Clinicopathologic features of primary leiomyosarcoma in central nervous system
周婧 1杨国仪 1李南云 2周志毅 1杨树东1
作者信息
- 1. 南京医科大学附属无锡人民医院病理科,无锡,214023
- 2. 南京大学医学院临床学院、南京军区南京总医院病理科,南京,210002
- 折叠
摘要
Abstract
Purpose To stuffy clinicopathologic features, diagnosis, treatment and prognosis of rare primary leiomyosarcoma in the central nervous system ( PLCNS ). Methods A case of PLCNS was studied by light microscopy, immunohistochemical and in situ hybridization. Based on the present case and 24 reported cases in English literature, clinicopathologic features, treatment and prognosis of PLCNS were analyzed. Results Reported a case of one-year and eight-month old child without specific findings suggestive of HIV infection. Magnetic resonance imaging of the brain revealed a mass in the right cerebellum. A craniotomy was performed, and the mass was resected. Microscopic examination showed highly pleomorphic, poorly differentiated spindle cells in a myxoid matrix and arranged in cords with abundant mitotic figures. Immunohistochemical examination showed that SMA was positive. Ki-67 proliferation index was about 80%. The tumor was EBV negative by in situ hybridization. In the 25 cases of PLCNS, 13 cases were immunosuppression and EBV coinfection-related, one case was radiation-related and the other 8 cases were without any special etiology. Conclusions PLCNS is a very rare tumor, which usually occurs in children and young adults. The neoplasm is more frequently associated with immunosuppression and EBV coinfection in young adults. This neoplasm typically present a supratentorial mass, but neoplasm associated with immunosuppression and EBV coinfection may occur in spinal cord or present multiple leiomyosarcomas of the central nervous system. The imaging characteristic of the neoplasm is a large mass with mild edema. The histopathology of neoplasm is frequently well differentiated, while the neoplasm without any special etiology are always poorly-differentiated leiomyosarcoma, including myxoid leiomyosarcoma. PLCNS shows poor prognosis and surgical resection is the main treatment. The role of adjuvant therapy in the management of the lesion is uncertain.关键词
中枢神经系统肿瘤/平滑肌肉瘤/临床病理/诊断/鉴别诊断Key words
central nervous system neoplasms/ leiomyosarcoma/ clinicalpathology/ diagnosis/ differential diagnosis分类
医药卫生引用本文复制引用
周婧,杨国仪,李南云,周志毅,杨树东..原发性中枢神经系统平滑肌肉瘤的临床病理分析[J].临床与实验病理学杂志,2012,28(9):1006-1011,6.
