Anghileri Elena, Schettino Carla, Pollo Bianca, Farinotti Mariangela, Silvani Antonio, Paterra Rosina, Patanè Monica, DiMeco Francesco, Bruzzone Maria Grazia, Eoli Marica, Cuccarini Valeria
Molecular Neuro-Oncological Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Department of Neuropathology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Neurol Sci. 2020 Aug;41(8):2111-2120. doi: 10.1007/s10072-020-04288-7. Epub 2020 Feb 29.
Gliomatosis cerebri (GC), defined until 2016 as a distinct astrocytic glioma entity, has been removed from the 2016 World Health Organization classification of tumors of the central nervous system. However, its identity is still debated.
We retrospectively present 122 patients, including a subgroup with histology confirmation (n = 75, cohort b).
Radiological features showed extension limited to 3 lobes in 31%; bilateral, midline, and basal ganglia and subtentorial involvement in 95%, 52%, 84%, and 60%, respectively; and contrast enhancement in 59.5%. Perioperative mortality occurred in 4%. Histology concluded for grades II, III, and IV, respectively, in 31%, 35%, and 22% (not specified in 12%). Thirty-one percent had isocitrate dehydrogenase (IDH) 1 mutation. Treatments included radiotherapy in 51.2% and chemotherapy in 74.5%. Median overall survival was 17 months. Negative prognostic factors for survival were older age, poorer Karnofsky Performance Scale (KPS), subtentorial, midline and disseminated disease, and lack of chemotherapy, at univariate analysis. At multivariate analysis, KPS ≥ 80, chemotherapy, and subtentorial and disseminated disease remained prognostic (p < 0.0001). For cohort b, same prognostic factors were confirmed, except for midline location, at univariate analysis; at multivariate analysis, only KPS ≥ 80 and chemotherapy remained prognostic (p < 0.0001).
We described clinical, neuroimaging, management, and histomolecular features of one of the largest GC series. We identified KPS ≥ 80, radiological pattern as subtentorial localization and dissemination, and chemotherapy as prognostic factors, at multivariate analysis. Planning prospective study, associated to focused genetic assays, could help to clarify if GC has specific features that may result in the identification as a separate entity from other gliomas.
大脑胶质瘤病（GC）在2016年之前被定义为一种独特的星形胶质细胞瘤实体，现已从2016年世界卫生组织中枢神经系统肿瘤分类中移除。然而，其身份仍存在争议。
我们回顾性地呈现了122例患者，包括一个有组织学确诊的亚组（n = 75，队列b）。
放射学特征显示，31%的患者病变范围局限于3个脑叶；双侧、中线、基底节和幕下受累的比例分别为95%、52%、84%和60%；59.5%的患者有对比增强。围手术期死亡率为4%。组织学结果显示，分别有31%、35%和22%的患者为二级、三级和四级（12%未明确分级）。31%的患者有异柠檬酸脱氢酶（IDH）1突变。治疗方法包括51.2%的患者接受放疗，74.5%的患者接受化疗。中位总生存期为17个月。单因素分析显示，生存的负性预后因素包括年龄较大、卡氏功能状态评分（KPS）较低、幕下、中线和播散性疾病以及未接受化疗。多因素分析显示，KPS≥80、化疗以及幕下和播散性疾病仍具有预后意义（p < 0.0001）。对于队列b，单因素分析确认了相同的预后因素，但中线位置除外；多因素分析显示，只有KPS≥80和化疗仍具有预后意义（p < 0.0001）。
我们描述了最大的GC系列之一的临床、神经影像学、治疗和组织分子特征。多因素分析显示，我们确定KPS≥80、放射学表现为幕下定位和播散以及化疗为预后因素。规划前瞻性研究并结合针对性的基因检测，可能有助于明确GC是否具有特定特征，从而使其被认定为与其他胶质瘤不同的独立实体。
