H3K27M突变型弥漫性中线胶质瘤的诊治分析

Clinical analysis of H3K27M-mutant diffuse midline glioma:report of 11 cases and review of literature

目的总结H3K27M突变型弥漫性中线胶质瘤(HM-DMG)的诊治经验。方法回顾性分析2017年9月至2020年9月手术治疗的11例HM-DMG的临床资料。术后随访至2020年9月30日。结果11例中,肿瘤全切除1例,次全切除6例,部分切除4例。术后病理确诊为HM-DMG,WHO分级Ⅳ级10例,Ⅱ级1例。1例术后6个月复发,二次手术;4例术后行规律放、化疗,5例只化疗,1例未行放化疗。术后1个月内死亡1例,6~12个月死亡7例,12~18个月死亡1例。术后存活28 d~13个月,生存曲线分析显示,累积中位生存时间10.1个月。结论HM-DMG属于高级别胶质瘤,确诊有赖于病理检查。即使采用手术和辅助术后放化疗等综合治疗,病人预后仍较差。

Objective To summarize the experience in diagnosis and treatment of the patients with H3K27M-mutant diffuse midline glioma(HM-DMG).Methods The clinical data of 11 patients with HM-DMG were analyzed retrospectively.The diagnosis,treatment and outcomes were analyzed.The deadline of postoperative follow-up was on September 30th,2020.Results Of 11 patients with HM-DMG,total tumor resection was achieved in 1,subtotal in 6 and partial in 4.The postoperative pathological examination showed HM-DMG in all the patients,with WHO gradeⅣin 10 patients and gradeⅡin 1.Of 11 patients,1 patient relapsed 6 months after the surgery and received a second operation,4 received regular radiotherapy and chemotherapy after the operation,5 received only chemotherapy,and 1 did not receive radiotherapy or chemotherapy;1 died within 1 month after the operation,7 within 6~12 months,and 1 within 12~18 months.The postoperative survival ranged from 28 days to 13 months.The survival curve analysis showed that the cumulative median survival time was 10.1 months.Conclusions HM-DMG,whose difinite diagnosis depends on the pathological examination,belongs to high-grade glioma.The prognoses of HM-DMG patients are poor despite surgery and adjuvant postoperative radiotherapy and chemotherapy.