大脑半球胶质肉瘤的临床病理观察

Gliosarcoma of cerebral hemispheres： a clinicopathologic study of 10 cases

目的 分析大脑半球胶质肉瘤病例的临床病理特征.方法 收集10例大脑半球胶质肉瘤病例,通过光镜下观察、免疫组织化学EnVision法及组织化学染色检测分析其临床病理特征及免疫组织化学和组织化学特点.结果 10例胶质肉瘤患者平均年龄54岁,男6例,女4例,临床表现以头痛（6/10）、恶心呕吐（5/10）及肢体感觉障碍或活动障碍（4/10）为主.9例为原发性,均为单发占位性病变,肿物最大径2.4～5.5 cm（平均4.2 cm）;1例为继发性,病变广泛累及颅底及颅外.术中见肿瘤距皮层1.5 ～4.0 cm（平均2.4 cm）,4例边界不清.镜下均见2种成分.6例术后有随访,5例随访3～17个月死亡（平均生存9个月）,1例随访45个月无复发,4例失访;免疫组织化学胶质纤维酸性蛋白（GFAP,10/10）、p53（6/10）、p16（4/10）、表皮生长因子受体（EGFR,1/10）、CD68（1/10）阳性表达,10例Ki-67阳性指数15％ ～70％,平均34％;组织化学染色网织纤维均阳性.结论 胶质肉瘤主要位于大脑实质深部,肿瘤生长快,病程短,临床发现时肿瘤病灶已很大.免疫组织化学GFAP结合组织化学网织纤维染色有助于胶质母细胞瘤和肉瘤两种成分的确定.部分病例p53、p16阳性表达,EGFR罕见表达.病变广泛,手术不易切净,预后较差.

Objective To study the clinical and pathologic features of gliosarcoma of cerebral hemispheres.Methods The clinicopathologic features of 10 cases of gliosarcoma involving cerebral hemispheres were reviewed.Immunohistochemical study was carried out using EnVision method.Results The mean age of the patients was 54 years and the male-to-female ratio was 6 to 4.Clinical symptoms included headache （6/10）,nausea / vomiting （5/10）,and sensory or motor impairment （4/10）.Nine of the cases were primary gliosarcoma,with maximum diameter ranging from 2.4 to 5.5 cm （mean =4.2 cm）.The remaining case represented secondary gliosarcoma involving skull base and extracranial tissues.Histologic examination showed a biphasic pattern in all cases.Regarding the glial component,there were 9 cases of pleomorphic glioblastoma and 1 case of giant cell glioblastoma.Reticulin stain was positive in all cases.Immunohistochemical study showed that the tumor cells variably expressed GFAP （10/10）,p16 （4/10）,EGFR （1/10）,CD68 （1/10） and p53 （6/10）.The Ki-67 index ranged from 15％ to 70％ （mean =34％）.Six patients had follow-up data available.One patient was disease-free for 45 months and 5 patients died of the disease at 3 to 17 months after the operation （mean duration of survival =9 months）.Conclusions Gliosarcoma is a highly aggressive tumor,often locates in the deeper part cerebral hemispheres and has a relatively short duration of symptoms.It carries a poor prognosis.GFAP immunostain and reticulin stain are helpful in confirming the diagnosis.p53 and p16 are also expressed in some cases.