关键词: Five-year survival Medulloblastoma Molecular features Progression-free survival Radiological features

Mesh : Humans Medulloblastoma / diagnostic imaging genetics pathology mortality Retrospective Studies Child Male Female Child, Preschool Cerebellar Neoplasms / diagnostic imaging genetics pathology Prognosis Adolescent Magnetic Resonance Imaging Kuwait / epidemiology Progression-Free Survival Infant Survival Rate

来  源:   DOI:10.1016/j.wneu.2024.04.057

Abstract:
BACKGROUND: Medulloblastomas are the most common malignant brain tumors in the pediatric population. Based on the idea that tumors with identical radio-genomic features should behave similarly, the 4 molecular subtypes are now widely accepted as a guide for the management and prognosis. The radiological features of medulloblastomas can predict the molecular subtype; thus, anticipating the subsequent disease progression. However, this has not been evaluated comprehensively. We aim to thoroughly study the association between the molecular subtypes and radiological features of medulloblastomas. Moreover, we aim to investigate the efficacy of this correlation with the use of progression-free survival and 5-year survival rates.
METHODS: A retrospective analysis was conducted for all histopathological confirmed medulloblastomas in pediatric patients (<16 years old) that were operated on in Kuwait over the past ten years (n = 44). The radiological, histological, and molecular characteristics were justifiably evaluated and analyzed in our sample.
RESULTS: The overall progression-free survival after one year was noticed among 27 cases (≈44%) and the nonspecific 5-year survival was seen in 31 cases (≈70%) after a 5-year follow-up. Sonic Hedgehog and Wingless had the best outcomes, while group 3 showed the worst outcomes.
CONCLUSIONS: Our findings did not support the association between most of the typical magnetic resonance imaging characteristics and survival rate. We further established that Sonic Hedgehog and Wingless biological types have a better prognosis. There was no association observed between the radiographic features, specifically the location, and the molecular subtype.
摘要:
背景:髓母细胞瘤是儿科人群中最常见的恶性脑肿瘤。基于具有相同放射基因组特征的肿瘤应该表现相似的想法,这四种分子亚型现在被广泛接受为治疗和预后指南.髓母细胞瘤的放射学特征可以预测分子亚型;因此,预测随后的疾病进展。然而,这还没有得到全面评估。
目的:我们旨在深入研究髓母细胞瘤的分子亚型与影像学特征之间的关联。此外,我们的目的是研究这种相关性与无进展生存期(PFS)和5年生存率的疗效.
方法:对过去10年在科威特接受手术的所有经组织病理学证实的髓母细胞瘤患儿(<16岁)进行回顾性分析(n=44)。放射学,组织学,和分子特征在我们的样本中进行了合理的评估和分析。
结果:观察到27例患者一年后的总体无进展生存期(≈44%),31例非特异性五年生存率(≈70%)随访5年。SHH和WNT有最好的结果,而第3组的结局最差。
结论:我们的发现不支持大多数典型MRI特征与生存率之间的关联。我们进一步确定SHH和WNT生物类型具有更好的预后。射线照相特征之间没有观察到关联,特别是位置,和分子亚型。
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