成人小脑髓母细胞瘤的临床及病理研究

目的探讨成人小脑髓母细胞瘤的临床病理特点、组织发生学及影响预后的因素。方法对我院１９７４～１９９５年间２７例成年人（≥１６岁）小脑髓母细胞瘤进行了观察。结果本组年龄１６～５５岁，平均２５．８岁，肿瘤发生于小脑蚓部２１例，小脑半球６例。随访２１例，７例已生存２．５～１７年，１４例死亡。平均生存时间４１．６个月。１年生存率为７１．４％，５年及１０年生存率均１４．３％。组织学类型：经典型１４例，促纤维增生型１３例。结论通过电镜观察及突触素、神经元特异性烯醇化酶和胶质纤维酸性蛋白免疫组化染色观察，证实髓母细胞瘤是具有向神经元及胶质细胞双向分化潜能的原始神经外胚叶肿瘤。影响预后的因素包括年龄、肿瘤部位、治疗手段以及组织学类型

A clinical and pathological study of cerebellar medulloblastoma in adult patients

OBJECTIVE: In order to study the clinicopathological characteristics, histogenesis and prognosis of medulloblastoma in the adult patients. METHODS: Altogether 27 patients of medulloblastoma admitted form 1974-1995 were reviewed. RESULTS: In all of the cases, age ranged from 16 to 55, (average 25.8 years) with 21 tumors localized in the cerebellar vermis, and 6 tumors in the cerebellum. Among them, 12 cases were completely while 14 cases were subtotally removed and biopsy was done in only one case. In the 21 patients to be followed up, 7 survived for 2.5-17 years after the operation, and 14 died. The average survival period was 41.6 months. One year survival rate was 71.4%, five years and ten years survival rates were 14.3% respectively. Regarding to the histological types, 14 cases were considered to be classic and 13 cases were desmoplastic by histological classification. CONCLUSION: Medulloblastoma keeps still the ability of differentiation to neurons and astrocytes confirmed by electronmicroscopy and immunohistochemical staining for Syn, NSE and GFAP. Factors considered responsible to the prognosis included age, tumor location, various therapeutic measurements and the histological classification.