Primary intracranial gliosarcoma is a rare malignant brain tumour, and the most effective treatment for gliosarcoma remains unclear. This study aimed to identify risk factors for progression-free survival (PFS) and overall survival (OS) in these cases. This retrospective single-centre study evaluated 103 patients (median age, 51 years; 67 men [65%]) with primary intracranial gliosarcoma between 2006 and 2017. Treatments included surgery (GTR, 63 patients; STR, 39 patients; biopsy, 1 patient), radiotherapy (adjuvant, 76 patients; exclusive treatment, 1 patient), and chemotherapy (adjuvant temozolomide, 52 patients; adjuvant nimustine/teniposide, 19 patients; adjuvant bevacizumab, 1 patient; exclusive nimustine/teniposide treatment, 1 patient). The median OS was 13.3 months, and the median PFS was 9.1 months. In the multivariate analyses, the poor prognostic factors were ependymal lining enhancement of the lateral ventricle (PFS, HR 2.406, p = 0.005; OS, HR 2.946, p = 0.009) and enhancement in the motor functional cortex (PFS, HR 2.892, p = 0.002; OS, HR 2.639, p = 0.009). Good OS was predicted by adjuvant radiotherapy alone (HR 0.071, p < 0.001), adjuvant temozolomide-based chemotherapy alone (HR 0.063, p = 0.005), adjuvant temozolomide-based chemotherapy with concurrent radiotherapy (HR 0.056, p < 0.001), and salvage surgery at recurrence (HR 0.449, p = 0.031). The present study revealed that, in patients with primary intracranial gliosarcoma, enhancement in the functional motor cortex and ependymal lining enhancement of the lateral ventricle were both poor prognostic factors. Survival was optimized in cases treated using maximal safe resection followed by adjuvant temozolomide-based chemotherapy with concurrent radiotherapy. Furthermore, salvage surgery provided meaningful therapeutic benefits for recurrent gliosarcoma.

原发性颅内神经胶质肉瘤是一种罕见的恶性脑肿瘤，目前尚不清楚最有效的治疗方法。这项研究旨在确定这些情况下无进展生存期（PFS）和总体生存期（OS）的危险因素。这项回顾性单中心研究评估了2006年至2017年间103例原发性颅内神经胶质肉瘤患者（中位年龄51岁； 67名男性[65％]）。治疗方法包括手术（GTR，63例； STR，39例；活检，1例） ），放疗（辅助治疗76例;独家治疗1例）和化学疗法（替莫唑胺辅助52例;尼莫司汀/替尼泊甙辅助19例;贝伐单抗辅助1例;尼莫斯汀/替尼泊甙独家治疗1例）。中位OS为13.3个月，中位PFS为9.1个月。在多元分析中p  = 0.005；OS，HR 2.946，p  = 0.009）和运动功能皮质增强（PFS，HR 2.892，p  = 0.002； OS，HR 2.639，p  = 0.009）。单独的辅助放疗（HR 0.071，p  <0.001），单独的基于替莫唑胺的辅助化疗（HR 0.063，p  = 0.005），基于替莫唑胺的辅助化疗并发放疗（HR 0.056，p  <0.001）和复发时的抢救手术（HR 0.449，p = 0.031）。本研究表明，在原发性颅内神经胶质肉瘤患者中，功能性运动皮层的增强和侧脑室的室管膜衬层的增强都是不良的预后因素。使用最大安全切除术，基于替莫唑胺的辅助化疗和同步放疗治疗的患者，可以优化生存率。此外，抢救手术为复发性青光眼肉瘤提供了有意义的治疗益处。