Abstract：
OBJECTIVE: To systematically review the clinical features, management, and outcomes of diffuse midline H3K27-altered gliomas of the spinal cord (DMG-SCs).
METHODS: PubMed, Ovid EMBASE, Scopus, and Web of Science were searched from database inception to 23 September 2023 for histologically confirmed cases of DMG-SC. Patient demographics, tumor characteristics, management information, and survival outcomes were extracted and analyzed.
RESULTS: A total of 279 patients from 39 studies were collected. Patients were mostly male (61%), with an average age of 32 years. Patients were treated with surgery, radiotherapy, and chemotherapy combined (31%) or surgery only (24%), and extent of resection was most often subtotal (38%). Temozolomide was the most common chemotherapeutic agent (81%). Radiation therapy was delivered with mean dose of 47 Gy in 23 fractions. At mean follow-up time of 21 months, 13% of patients were alive. Average median overall survival was 24 months (range of 13 to 40 months) with a median progression-free survival of 14 months. Historical WHO grades of 2 or 3 appeared to exhibit a longer average median overall survival time than that of grade 4 DMG-SCs (32 vs. 23 months, p = 0.009).
CONCLUSIONS: Outcomes for DMG-SCs are poor overall but appear to be favorable compared to intracranial DMGs. Despite the recent WHO 2021 grade 4 classification for all DMGs, given the differences in overall survival reported based on historical grading systems, future studies on DMG-SCs are needed to further define if DMG-SCs may represent a heterogeneous group of tumors with different prognoses.
METHODS: PubMed, Ovid EMBASE, Scopus, and Web of Science were searched from database inception to 23 September 2023 for histologically confirmed cases of DMG-SC. Patient demographics, tumor characteristics, management information, and survival outcomes were extracted and analyzed.
RESULTS: A total of 279 patients from 39 studies were collected. Patients were mostly male (61%), with an average age of 32 years. Patients were treated with surgery, radiotherapy, and chemotherapy combined (31%) or surgery only (24%), and extent of resection was most often subtotal (38%). Temozolomide was the most common chemotherapeutic agent (81%). Radiation therapy was delivered with mean dose of 47 Gy in 23 fractions. At mean follow-up time of 21 months, 13% of patients were alive. Average median overall survival was 24 months (range of 13 to 40 months) with a median progression-free survival of 14 months. Historical WHO grades of 2 or 3 appeared to exhibit a longer average median overall survival time than that of grade 4 DMG-SCs (32 vs. 23 months, p = 0.009).
CONCLUSIONS: Outcomes for DMG-SCs are poor overall but appear to be favorable compared to intracranial DMGs. Despite the recent WHO 2021 grade 4 classification for all DMGs, given the differences in overall survival reported based on historical grading systems, future studies on DMG-SCs are needed to further define if DMG-SCs may represent a heterogeneous group of tumors with different prognoses.
摘要：
目的：系统评价临床特征，管理,和弥漫性中线H3K27改变的脊髓神经胶质瘤（DMG-SCs）的结果。
方法：PubMed，OvidEmbase,Scopus,和WebofScience从数据库开始至2023年9月23日检索经组织学证实的DMG-SC病例。患者人口统计学，肿瘤特征，管理信息，和生存结局进行提取和分析。
结果：共收集了来自39项研究的279名患者。患者多为男性（61%），平均年龄32岁。患者接受手术治疗，放射治疗,联合化疗(31%)或仅手术(24%)，切除程度最常见(38%)。替莫唑胺是最常见的化学治疗剂(81%)。放射治疗的平均剂量为47Gy，分为23个部分。平均随访21个月,13%的患者还活着。平均中位总生存期为24个月（13至40个月），中位无进展生存期为14个月。历史上WHO2级或3级似乎比4级DMG-SCs表现出更长的平均中位总生存时间（32vs.23个月,p=0.009)。
结论：与颅内DMG相比，DMG-SCs的结果总体较差，但似乎是有利的。尽管最近对所有DMG进行了WHO2021年的4级分类，考虑到基于历史分级系统报告的总生存率差异，需要对DMG-SCs进行进一步研究,以进一步确定DMG-SCs是否代表具有不同预后的异质性肿瘤组.
方法：PubMed，OvidEmbase,Scopus,和WebofScience从数据库开始至2023年9月23日检索经组织学证实的DMG-SC病例。患者人口统计学，肿瘤特征，管理信息，和生存结局进行提取和分析。
结果：共收集了来自39项研究的279名患者。患者多为男性（61%），平均年龄32岁。患者接受手术治疗，放射治疗,联合化疗(31%)或仅手术(24%)，切除程度最常见(38%)。替莫唑胺是最常见的化学治疗剂(81%)。放射治疗的平均剂量为47Gy，分为23个部分。平均随访21个月,13%的患者还活着。平均中位总生存期为24个月（13至40个月），中位无进展生存期为14个月。历史上WHO2级或3级似乎比4级DMG-SCs表现出更长的平均中位总生存时间（32vs.23个月,p=0.009)。
结论：与颅内DMG相比，DMG-SCs的结果总体较差，但似乎是有利的。尽管最近对所有DMG进行了WHO2021年的4级分类，考虑到基于历史分级系统报告的总生存率差异，需要对DMG-SCs进行进一步研究,以进一步确定DMG-SCs是否代表具有不同预后的异质性肿瘤组.
