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胶质肉瘤的临床特征及相对于传统胶质母细胞瘤的标准化治疗结果

Clinical Characteristics of Gliosarcoma and Outcomes From Standardized Treatment Relative to Conventional Glioblastoma.

作者信息

Frandsen Simone, Broholm Helle, Larsen Vibeke Andrée, Grunnet Kirsten, Møller Søren, Poulsen Hans Skovgaard, Michaelsen Signe Regner

机构信息

Department of Radiation Biology, Rigshospitalet, Copenhagen, Denmark.

Department of Pathology, Rigshospitalet, Copenhagen, Denmark.

出版信息

Front Oncol. 2019 Dec 17;9:1425. doi: 10.3389/fonc.2019.01425. eCollection 2019.

Abstract

Gliosarcoma (GS) is a rare histopathologic variant of glioblastoma (GBM) characterized by a biphasic growth pattern consisting of both glial and sarcomatous components. Reports regarding its relative prognosis compared to conventional GBM are conflicting and although GS is treated as conventional GBM, supporting evidence is lacking. The aim of this study was to characterize demographic trends, clinical outcomes and prognostic variables of GS patients receiving standardized therapy and compare these to conventional GBM. Six hundred and eighty GBM patients, treated with maximal safe resection followed by radiotherapy with concomitant and adjuvant temozolomide at a single institution, were retrospectively reevaluated by reviewing histopathological records and tumor tissue for identification of GS patients. Clinico-pathological- and tumor growth characteristics were obtained via assessment of medical records and imaging analysis. Kaplan-Meier survival estimates were compared with log-rank testing, while Cox-regression modeling was tested for prognostic factors in GS patients. The cohort included 26 primary gliosarcoma (PGS) patients (3.8%) and 7 secondary gliosarcoma (SGS) patients (1.0%). Compared to conventional GBM tumors, PGS tumors were significantly more often MGMT-unmethylated (73.9%) and located in the temporal lobe (57.7%). GS tumors often presented dural contact, while extracranial metastasis was only found in 1 patient. No significant differences were found between PGS and conventional GBM in progression-free-survival (6.8 and 7.6 months, respectively, = 0.105) and in overall survival (13.4 and 15.7 months, respectively, = 0.201). Survival following recurrence was not significantly different between PGS, SGS, and GBM. Temporal tumor location and MGMT status were found associated with PGS survival ( = 0.036 and = 0.022, respectively). Despite histopathological and location difference between GS and GBM tumors, the patients present similar survival outcome from standardized treatment. These findings support continued practice of radiation and temozolomide for GS patients.

摘要

胶质肉瘤(GS)是胶质母细胞瘤(GBM)一种罕见的组织病理学变体,其特征为具有由神经胶质和肉瘤成分组成的双相生长模式。关于其与传统GBM相比的相对预后的报道相互矛盾,尽管GS被当作传统GBM进行治疗,但缺乏支持证据。本研究的目的是描述接受标准化治疗的GS患者的人口统计学趋势、临床结局和预后变量,并将这些与传统GBM进行比较。对在单一机构接受最大安全切除,随后进行放疗并同步和辅助使用替莫唑胺治疗的680例GBM患者,通过回顾组织病理学记录和肿瘤组织以识别GS患者进行回顾性重新评估。通过评估病历和影像分析获得临床病理及肿瘤生长特征。采用对数秩检验比较Kaplan-Meier生存估计值,同时对GS患者的预后因素进行Cox回归建模检验。该队列包括26例原发性胶质肉瘤(PGS)患者(3.8%)和7例继发性胶质肉瘤(SGS)患者(1.0%)。与传统GBM肿瘤相比,PGS肿瘤中O6-甲基鸟嘌呤-DNA甲基转移酶(MGMT)未甲基化的情况显著更常见(73.9%),且多位于颞叶(57.7%)。GS肿瘤常表现为与硬脑膜接触,而仅在1例患者中发现颅外转移。PGS与传统GBM在无进展生存期(分别为6.8个月和7.6个月,P = 0.105)和总生存期(分别为13.4个月和15.7个月,P = 0.201)方面未发现显著差异。PGS、SGS和GBM复发后的生存期无显著差异。发现肿瘤位于颞叶和MGMT状态与PGS生存期相关(分别为P = 0.036和P = 0.022)。尽管GS和GBM肿瘤在组织病理学和位置上存在差异,但患者经标准化治疗后的生存结局相似。这些发现支持继续对GS患者采用放疗和替莫唑胺治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c53/6928109/f4cfc3b4af92/fonc-09-01425-g0001.jpg

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