Ntenti Charikleia, Lallas Konstantinos, Papazisis Georgios
First Department of Pharmacology, School of Medicine, Aristotle University of Thessaloniki, 54621 Thessaloniki, Greece.
Department of Medical Oncology, School of Medicine, Aristotle University of Thessaloniki, 54621 Thessaloniki, Greece.
Diagnostics (Basel). 2023 May 30;13(11):1915. doi: 10.3390/diagnostics13111915.
Medulloblastomas, highly aggressive neoplasms of the central nervous system (CNS) that present significant heterogeneity in clinical presentation, disease course, and treatment outcomes, are common in childhood. Moreover, patients who survive may be diagnosed with subsequent malignancies during their life or could develop treatment-related medical conditions. Genetic and transcriptomic studies have classified MBs into four subgroups: wingless type (WNT), Sonic Hedgehog (SHH), Group 3, and Group 4, with distinct histological and molecular profiles. However, recent molecular findings resulted in the WHO updating their guidelines and stratifying medulloblastomas into further molecular subgroups, changing the clinical stratification and treatment management. In this review, we discuss most of the histological, clinical, and molecular prognostic factors, as well the feasibility of their application, for better characterization, prognostication, and treatment of medulloblastomas.
髓母细胞瘤是中枢神经系统（CNS）中极具侵袭性的肿瘤，在临床表现、疾病进程和治疗结果方面存在显著异质性，在儿童期较为常见。此外，存活下来的患者在其一生中可能被诊断出患有后续恶性肿瘤，或者可能出现与治疗相关的医疗状况。遗传和转录组学研究已将髓母细胞瘤分为四个亚组：无翼型（WNT）、音猬因子（SHH）、3组和4组，各有不同的组织学和分子特征。然而，最近的分子研究结果促使世界卫生组织更新了其指南，并将髓母细胞瘤进一步细分为分子亚组，改变了临床分层和治疗管理。在本综述中，我们讨论了大多数组织学、临床和分子预后因素及其应用的可行性，以便更好地对髓母细胞瘤进行特征描述、预后评估和治疗。
