胶质肉瘤的临床特征及随访分析：13例病例总结

目的探讨胶质肉瘤（GSM）的临床特征及术后随访情况。方法回顾性分析2012年8月—2020年7月于贵州医科大学附属医院神经外科就诊并行开颅手术的13例GSM患者的临床资料，包括基本临床特征、术前影像学特征及术后分子病理表现。术后进行随访，直至患者死亡。结果GSM患者共13例，男8例，女5例；年龄 23~70岁，平均51.69岁；大部分患者主诉为头晕、头痛，病灶主体位于幕上。术前核磁共振成像提示主要为团块状，T1WI 、T2WI大部分为混杂密信号，T1增强提示环形强化或者不规则强化。术后病理诊断示，病灶均含有胶质母细胞瘤成分和肉瘤成分，网织纤维染色为阳性。分子免疫学提示胶质纤维酸性蛋白、波形蛋白、S-100蛋白、少突胶质细胞转录因子蛋白均为阳性。术后3例行放疗，8例行替莫唑胺化疗，随访发现复发5例，其中3例原发病灶复发，1例经脑脊液颅外转移，1例患者转移至延髓、第四脑室。总体中位数生存期9.20个月。结论GSM罕见，主要依靠病理特征确诊，容易复发，一般向邻近脑组织和自然解剖途径转移，预后极差。

Objective To investigate the clinical characteristics and postoperative follow-up of gliosarcoma（GSM）. Methods The clinical data of 13 patients with GSM who underwent surgery at the Affiliated Hospital of Guizhou Medical University from August 2012 to July 2020 were retrospectively analyzed, including basic clinical features, preoperative imaging features, and postoperative molecular pathological findings. Follow-up was performed, until the patient died. Results 8 male and 5 female patients with GSM were collected. The average age was 51.69 years old, ranging from 23 to 70 years old. The chief complaints of most patients were dizziness and headache, and the mainly located on the supratentorial region. Preoperative MRI showed that the lesions were mainly lumpy in T1WI and T2WI. T1 enhanced MRI revealed annular enhancement or irregular enhancement. All the pathological diagnosis after operation indicated both glioblastoma components and sarcoma components, and reticulofibers staining was positive, staining by positive cells of glial fibrillary acidic protein, vimentin, S-100 and Olig-2. Postoperative radiotherapy was 3 cases and temozolomide chemotherapy was 8 cases. Postoperative follow-up revealed recurrence in 5 cases, including 3 of primary focus recurrence, 1 of extracranial metastasis via cerebrospinal fluid, and 1 of metastasis to medulla oblongata and the fourth ventricle, with 8.47 months of the overall median survival. Conclusions GSM，mainly diagnosed by pathological features, was rare and relapsed easily. Generally, GSM transfers to adjacent brain tissues and metastasized natural anatomical pathways, with poor prognosis.