Department of Pediatrics, British Columbia Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Department of BioMedical Research, University of Bern, Bern, Switzerland.
Pediatr Dev Pathol. 2021 Jul-Aug;24(4):309-317. doi: 10.1177/10935266211001986. Epub 2021 Mar 22.
The World Health Organization currently classifies medulloblastoma (MB) into four molecular groups (WNT, SHH, Group 3 and Group 4) and four histologic subtypes (classic, desmoplastic nodular, MB with extensive nodularity, and large cell/anaplastic). "Classic" MB is the most frequent histology, but unfortunately it does not predict molecular group or patient outcome. While MB may exhibit additional histologic features outside of the traditional WHO subtypes, the clinical significance of such features, in a molecular context, is unclear.
The clinicopathologic features of 120 pediatric MB were reviewed in the context of NanoString molecular grouping. Each case was evaluated for five ancillary histologic features, including: nodularity without desmoplasia (i.e., "biphasic", B-MB), rhythmic palisades, and focal anaplasia. Molecular and histological features were statistically correlated to clinical outcome using Chi-square, log-rank, and multivariate Cox regression analysis.
While B-MB (N = 32) and rhythmic palisades (N = 12) were enriched amongst non-WNT/SHH MB (especially Group 4), they were not statistically associated with outcome. In contrast, focal anaplasia (N = 12) was not associated with any molecular group, but did predict unfavorable outcome.
These data nominate B-MB as a surrogate marker of Groups 3 and particularly 4 MB, which may earmark a clinically significant subset of cases.
世界卫生组织（WHO）目前将髓母细胞瘤（MB）分为四个分子组（WNT、SHH、第 3 组和第 4 组）和四个组织学亚型（经典型、促结缔组织增生性结节型、广泛结节型和大细胞/间变型）。“经典型”MB 是最常见的组织学类型，但不幸的是，它不能预测分子组或患者预后。虽然 MB 可能表现出超出传统 WHO 亚型的其他组织学特征，但这些特征在分子背景下的临床意义尚不清楚。
在 NanoString 分子分组的背景下，回顾了 120 例小儿 MB 的临床病理特征。评估了每种病例的五种辅助组织学特征，包括：无促结缔组织增生性结节的结节性（即“双相性”，B-MB）、节律性栅状结构和局灶性间变。使用卡方检验、对数秩检验和多变量 Cox 回归分析，对分子和组织学特征与临床结局进行统计学相关性分析。
B-MB（N=32）和节律性栅状结构（N=12）在非 WNT/SHH MB 中更为丰富（尤其是第 4 组），但与结局无统计学关联。相比之下，局灶性间变（N=12）与任何分子组均无关联，但与不良预后相关。
这些数据将 B-MB 作为第 3 组特别是第 4 组 MB 的替代标志物，可能标志着一组具有重要临床意义的病例。
