目的 探讨后颅窝室管膜瘤临床病理特征.方法 收集2022年10月-2023年10新疆医科大学第一附属医院病理科存档的后颅窝室管膜瘤共6例.调阅组织切片,分析免疫组化法检测相应指标表达情况,并收集随访信息,查阅相关文献总结其临床病理学表现.结果 6例后颅窝室管膜瘤中PFA组4例,PFB组2例.临床表现为头疼,呕吐,继发性脑积水等.PFA组患者均为儿童,年龄10个月-11岁,其中女性3例,男性1例;肿瘤3例位于四脑室,1例位于脑干.2例PFB组患者均为成年女性,年龄分别为32岁和45岁,肿瘤均位于四脑室.肿瘤组织学形态呈片状生长模式,出现血管周围假性菊形团,真性室管膜菊形团,组织学分级CNS WHO 2级1例,3级5例.6例后颅窝室管膜瘤免疫组化染色结果显示肿瘤细胞GFAP、S-100均弥漫性强阳性表达,EMA、D2-40表现为核旁点状及腔缘阳性,P53大部分为野生型,oligo-2、Neu-N均阴性.PFA组出现特征性H3 K27Me3细胞核表达缺失,Ki-67增殖指数20％-60％.PFB组H3 K27Me3细胞核未缺失,Ki-67增殖指数4％-15％.结论 后颅窝室管膜瘤PFA组好发于儿童且预后较差,通过H3K27me3免疫组化染色初筛并分组,对于明确诊断及指导临床辅助治疗具有重要临床意义.
Clinicopathological analysis of ependymoma in posterior cranial fossa
Objective To investigate the clinicopathological features of ependymoma in the posterior cranial fossa.Methods 6 cases of ependymoma in the posterior cranial fossa were collected from October 2022 to October 2023 in the Department of Pathology of the first affiliated Hospital of Xinjiang Medical University.The tissue sections were reviewed,the expression of corresponding indica-tors was detected by immunohistochemical method,follow-up information was collected,and its clinicopathological manifestations were summarized by the relevant literature.Results Among the 6 cases of ependymoma of posterior cranial fossa,there were 4 cases in PFA group and 2 cases in PFB group.The clinical manifestations were headache,vomiting,secondary hydrocephalus and so on.All the patients in PFA group were children,aged from 10 months to 11 years old,including 3 females and 1 male,3 tumors located in the fourth ventricle and 1 in the brain stem.Two patients in PFB group were adult women,aged 32 and 45 years old,and the tumors were located in the fourth ventricle.The histological morphology of the tumor showed a diffuse growth pattern,with perivascular pseudo ro-settes and true ependymal rosettes,histological grade CNS WHO 2 in 1 case and grade 3 in 5 cases.Immunohistochemical staining showed that the tumor cells were diffusely strongly positive for GFAP and S-100,EMA and D2-40 were positive for paranuclear dot-like or ring-like pattern,most of P53 were wild type,and oligo-2 and Neu-N were negative.The characteristic loss of nuclear expres-sion of H3 K27Me3 was found in the PFA group,and the Ki-67 proliferation index was 20％-60％.In PFB group,there was no nuclear deletion in H3 K27Me3,and the Ki-67 proliferation index was 4％-15％.Conclusion The PFA group of posterior fossae ependy-moma is common in children and the prognosis is poor.Screening and grouping by H3 K27me3 immunohistochemical staining have im-portant clinical significance for clear diagnosis and guidance of clinical adjuvant therapy.
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