Division of Neurosurgery, Department of Surgery, Hamilton General Hospital, McMaster University, 237 Barton Street East, Hamilton, Ontario, L8L 2X2, Canada.
Cancer Metastasis Rev. 2020 Mar;39(1):235-243. doi: 10.1007/s10555-020-09865-y.
Pediatric brain tumors are the leading cause of childhood cancer mortality with medulloblastoma (MB) representing the most frequent malignant tumor. Although standardization of therapy resulted in a 2-fold reduction in mortality in patients with MB by 2002, it became clear that further improvements in clinical outcome would require a deeper understanding of the biology of MB. Employing the four main molecular MB subgroups (Wnt, Shh, Group 3 and Group 4), a restratification into clinicogenomic risk categories quantified an unacceptable survival for the high-risk group, urging researchers to focus their efforts towards acquiring a greater biological understanding of these children. Advancing in parallel with the molecular characterization and understanding of pediatric MB is the clinicogenomic correlations giving rise to recommendations for neurosurgical care. While unique observations that distinct radiological patterns can be identified to inform the MB molecular subgroup preoperatively, current neurosurgical practice remains maximal safe surgical resection followed by risk-adapted provision of adjuvant therapy in the context of a clinical trial.
小儿脑肿瘤是儿童癌症死亡的主要原因,其中髓母细胞瘤(MB)是最常见的恶性肿瘤。尽管到 2002 年,通过对治疗的标准化,MB 患者的死亡率降低了近两倍,但显然要进一步提高临床疗效,就需要更深入地了解 MB 的生物学特性。通过采用四个主要的分子 MB 亚组(Wnt、Shh、Group 3 和 Group 4),对临床基因组风险类别进行重新分层,量化了高危组不可接受的生存率,促使研究人员努力深入了解这些患儿的生物学特性。随着对小儿 MB 的分子特征和认识的不断深入,临床基因组相关性也随之出现,为神经外科护理提供了建议。尽管有独特的观察表明,可以在术前识别出不同的影像学模式来提示 MB 分子亚组,但目前的神经外科实践仍然是最大限度地安全手术切除,然后根据临床试验提供适应性辅助治疗。