51例髓母细胞瘤临床病理分析

Pathologic and clinical features of medulloblastoma:analysis of 51 cases

目的分析髓母细胞瘤(MB)的临床病理特点,以提高对MB的认识。方法回顾性研究2005～2010年确诊的MB,分析其症状、病理改变、免疫组织化学和预后。33例通过电话或信件随访。结果男27例,女24例,年龄1～56岁(平均14.5岁)。常见症状为头痛(96%)、呕吐(88%)和行走不稳(62.7%)。肿瘤主要位于小脑半球、第四脑室和颅后窝。根据2007年WHO中枢神经系统肿瘤分类标准分为:21例促结缔组织增生/结节型,18例伴有广泛结节型,9例间变型,3例大细胞型。肿瘤组织细胞密集,多有核分裂,胞浆少,有的呈假菊形团排列。免疫组化检测该肿瘤表达CD99(12/14),S-100(6/14),胶质纤维酸性蛋白(GFAP)(9/14)和突触素(Syn)(5/14)。获得患者是否继续生存、有无复发、转移或死亡的随访信息。结论最常见的MB为促结缔组织增生/结节型,其次分别为伴有广泛结节型、间变型和大细胞型。大细胞和间变型MB患者预后差,如果患儿年龄小于3岁,手术切除程度不够,或有脑脊液、脊柱、幕上或全身转移,则预后更差。

Objective To get better recognition of medulloblastoma and diminish misdiagnosis,51 cases of medulloblastoma were analyzed by retrospective study. Methods 51 cases of medulloblastoma in our hospital from January 2005 to January 2010 were an-alyzed for their clinical symptoms, pathologic changes,immunohistochemical staining and prognosis. Clinical follow-up was available for 33 patients. Results There were 27 males and 24 females whose ages ranged from 1 to 56 years old（median 14.5 years）. The most common presenting symptoms were headache（96 %）, vomiting（88 % ） and unstable walking（62.7 %）. Tumors were mainly lo-cated in cerebellar hemisphere,fourth ventricle or posterior cranial fossa. According to the 2007 WHO classification of nervous sys-tem tumors,there were 21 desmoplastic type/nodules cases, 18 cases with extensive nodule formation of medulloblastoma, 9 cases were anaplastie medulloblastoma, and 3 cases were large cell type. Histologically, the tumor is very cellular, many mitoses,little cy- toplasm, and has the tendency to form clusters and rosettes. Immunohistochemieal findings included expression of CD99 （12/14）, S-100（6/14） ,GFAP（9/14） and Syn（5/14）. Follow-up information was available for 33 patients. Conclusion This study showed that the majority is Desmoplastic/Nodular MB variant. Anaplastic and Large Cell MB have poor prognosis, with a high degree of inva-siveness. Prognosis is worse if the child was less than 3 years old.