胶质肉瘤五例临床病理分析及文献复习

Five cases of gliosarcoma: clinicopathological analysis and a literature review

目的 探讨胶质肉瘤的临床特征、形态及免疫组化特点。方法 回顾性分析泰安市中心医院5例胶质肉瘤患者的临床资料,观察胶质肉瘤的病理形态特点及免疫表型。结果 4例胶质肉瘤患者为男性,为原发肿瘤,1例为女性,继发于颅内胶质母细胞瘤,平均年龄54岁。发病症状主要为头痛、恶心、视物模糊、肢体麻木感及活动障碍、言语不清等。形态学显示肿瘤有2种组织学形态,4例均以肉瘤成分为主,主要为纤维肉瘤,胶质成分与肉瘤成分交织生长,不易辨认,1例肉瘤成分把胶质成分分割成地图状,光镜下易于识别出2种成分,胶质成分为高级别。所有病例均显示表皮生长因子受体(-),4例P53(+);胶质成分均显示神经胶质纤维酸性蛋白(+)、Olig-2(+)、异柠檬酸脱氢酶-1(-);肉瘤成分均显示Vim(+),特染网织纤维围绕每个细胞。Ki-67阳性指数为30%~70%。5例术后均有随访,3例随访3~10个月死亡(平均生存6个月),2例随访4.5~21.0个月无复发。结论 胶质肉瘤是异柠檬酸脱氢酶-1野生型胶质母细胞瘤的一种亚型,同时具有胶质与肉瘤成分,神经胶质纤维酸性蛋白和网织纤维染色有助于确定胶质母细胞瘤和肉瘤2种成分,胶质肉瘤TP53突变的发生率很高,很少有表皮生长因子受体和异柠檬酸脱氢酶-1突变。

Objective To analyze the clinical, morphological, and immunohistochemical features of gliosarcoma (GS). Methods The clinical and pathologic findings for five cases of GS diagnosed at Taian City Central Hospital were evaluated. Hematoxylin and eosin staining, reticulin staining, and immunohistochemistry were performed on archival paraffin sections. Results Among the five patients, 4 were male and 1 was female;4 had primary GS, and 1 had secondary GS to intracranial glioblastoma. The average age was 54.2 years. The main symptoms were headache, nausea, blurred vision, numbness of limbs and dysphoria, indistinct speech and so on. Histologic examination showed a biphasic pattern in all cases, and 4 cases were mainly composed of sarcoma while the gliomatous component was intertwined with the sarcomatous component, so it was not easy to recognize the gliomatous component. In 1 case, the two components were clearly divided into two parts, so they could be easily recognized. All cases were negative for EGFR, and 4 cases were P53(+). The gliomatous component was positive for GFAP and Olig- 2, while the sarcomatous component was positive for Vim, with reticular fibers around each cell. The Ki- 67 index ranged from 30% to 70%. Five patients had follow-up data available. Two patients were diseasefree for 4.5-21.0 months and three patients died of the disease at 3 to 10 months after the operation (mean duration of survival of 6 months). Conclusions GS with distinct gliomatous and sarcomatous components is a distinct entity of IDH-1 wild-type glioblastoma. GFAP immunostaining and reticulin staining are helpful in confirming the diagnosis. The incidence of TP53 mutation in gliosarcoma is very high while there are few EGFR and IDH-1 mutations.