H3K27M突变型弥漫性中线胶质瘤的诊治分析

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。

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

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 grade Ⅳ in 10 patients and grade Ⅱ in 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.