Gelal M F, Rezanko T A, Sarp A F, Dirilenoğlu F, Güvenç G, Ölmezoğlu A
Department of Radiology, İzmir Katip Celebi University Atatürk Training and Research Hospital, İzmir, Turkey.
Department of Pathology, İzmir Katip Celebi University Atatürk Training and Research Hospital, İzmir, Turkey.
Clin Neuroradiol. 2016 Sep;26(3):329-40. doi: 10.1007/s00062-014-0366-7. Epub 2014 Dec 17.
Rhabdoid glioblastoma (rGB) is a recently described, highly aggressive brain tumor, in which glioblastoma (GB) is associated with a rhabdoid component. So far only 21 cases have been reported and its imaging findings have not been studied in detail. In this paper, we present 11 additional cases and aim to depict detailed magnetic resonance imaging (MRI) features of this tumor in comparison with the previous cases of rGBs and our cohort of typical GBs.
Retrospective evaluation of the glass slides of 249 GB patients disclosed 14 cases with rhabdoid morphology. Eleven of these cases with available MRI were included in the study. Immunohistochemistry was also performed. MRI and clinicopathologic findings were compared with those of previous rGBs and typical GBs.
(1) rGB is usually a large, well-delineated solid tumor with extensive necrosis, heterogeneous contrast enhancement, occasional hemorrhage, and cysts, (2) rGB may present at an older age than previously reported, but still in younger individuals compared with typical GB patients, (3) tumor dissemination in the form of leptomeningeal seeding and more rarely extracranial metastases is a feature of rGBs, although leptomeningeal seeding may not be as high as previously reported, (4) prognosis is poor in rGBs.
rGB is a new entity which has not yet appeared in the WHO classification of central nervous system (CNS) tumors. Understanding its clinical and imaging features may help to distinguish it from other tumors of CNS.
横纹肌样胶质母细胞瘤（rGB）是一种最近被描述的高度侵袭性脑肿瘤，其中胶质母细胞瘤（GB）与横纹肌样成分相关。迄今为止，仅报道了21例，其影像学表现尚未得到详细研究。在本文中，我们报告了另外11例病例，旨在描述该肿瘤详细的磁共振成像（MRI）特征，并与先前报道的rGB病例以及我们的典型GB病例队列进行比较。
对249例GB患者的玻片进行回顾性评估，发现14例具有横纹肌样形态。其中11例有可用MRI的病例被纳入研究。还进行了免疫组织化学检查。将MRI和临床病理结果与先前的rGB和典型GB的结果进行比较。
（1）rGB通常是一个大的、边界清晰的实性肿瘤，伴有广泛坏死、不均匀强化、偶发出血和囊肿；（2）rGB的发病年龄可能比先前报道的要大，但与典型GB患者相比仍为年轻个体；（3）rGB的特征是以软脑膜播散形式的肿瘤播散，较少见颅外转移，尽管软脑膜播散可能没有先前报道的那么高；（4）rGB的预后较差。
rGB是一种尚未出现在世界卫生组织中枢神经系统（CNS）肿瘤分类中的新实体。了解其临床和影像学特征可能有助于将其与其他CNS肿瘤区分开来。
