Aim: Glioblastoma (GBM) is one of the most aggressive neoplasms of the central nervous system with dismal survival. In recent years, different variants of GBM have been described in the literature. GBM with areas of neuroectodermal differentiation (GBM-PNET) is a relatively new entity in GBM. Presence of the neuroectodermal component increases the propensity of systemic dissemination as with other intracranial primitive neuroectodermal tumors (PNET). The optimal treatment for these patients remains a controversy, with authors reporting local radiotherapy to craniospinal irradiation and chemotherapy. We intend to analyze the pattern of care for GBM with neuroectodermal component.
Materials and Methods: We retrieved data of four patients with GBM-PNET treated in our institute; data were also retrieved from published series to derive treatment and outcome results.
Results: In this series, we report the outcome of a series of four patients of GBM-PNET treated with adjuvant radiotherapy and temozolomide. All but one patient underwent gross total resection of the tumor. Adjuvant hypofractionated radiation with concurrent and adjuvant temozolomide was used in all cases. The median follow-up was 12.9 months in the present series. One patient experienced local recurrence 18 months after the treatment. A review of published literature on GBM-PNET was done; studies with details of patient outcome were used for an independent analysis. Twenty-three patients were identified, and the pooled analysis revealed a median progression free and overall survival of 10 and 25, months respectively. Extent of surgery, local radiation vs. craniospinal irradiation, and age at presentation had no impact on the survival.
Conclusion: GBM PNET is a new entity with only few cases reported so far. Clinical behavior and treatment outcome of these tumors are not different from conventional GBM. However, these patients are at higher risk of CSF dissemination. Hence, an individualized treatment approach is best suited.


中文翻译：

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用辅助放疗和替莫唑胺治疗原始神经外胚层成分的胶质母细胞瘤：23 例患者的汇总分析


目的：胶质母细胞瘤（GBM）是中枢神经系统最具侵袭性的肿瘤之一，存活率低。近年来，文献中描述了 GBM 的不同变体。具有神经外胚层分化区域的 GBM (GBM-PNET) 是 GBM 中一个相对较新的实体。与其他颅内原始神经外胚层肿瘤 (PNET) 一样，神经外胚层成分的存在增加了全身传播的倾向。对这些患者的最佳治疗仍然存在争议，作者报告了局部放疗到颅脊椎放疗和化疗。我们打算分析带有神经外胚层成分的 GBM 的护理模式。
材料和方法：我们检索了在我院治疗的 4 名 GBM-PNET 患者的数据；还从已发表的系列中检索数据以得出治疗和结果结果。
结果：在本系列中，我们报告了接受辅助放疗和替莫唑胺治疗的四名 GBM-PNET 患者的结果。除一名患者外，所有患者均接受了肿瘤的大体全切除术。在所有病例中都使用了大分割放疗与并发和辅助替莫唑胺。本系列的中位随访时间为 12.9 个月。一名患者在治疗后 18 个月出现局部复发。对已发表的 GBM-PNET 文献进行了回顾；具有患者结果细节的研究用于独立分析。确定了 23 名患者，汇总分析显示中位无进展生存期和总生存期分别为 10 个月和 25 个月。手术范围、局部放射与颅脊椎放射以及就诊年龄对生存率没有影响。
结论： GBM PNET 是一个新实体，迄今为止报告的病例很少。这些肿瘤的临床行为和治疗结果与常规 GBM 没有区别。然而，这些患者的脑脊液传播风险较高。因此，个体化的治疗方法是最合适的。