Embryonal tumors

Cheshier, Samuel H.; Doz, François; Ellison, David W.; Merchant, Thomas E.; Pfister, Stefan M.

doi:10.1093/med/9780198869702.003.0013

Abstract

Embryonal tumors account for approximately 20% of pediatric central nervous system (CNS) tumors. Medulloblastoma (MB), the most frequent embryonal tumor type, arises in the cerebellum. Clinical strategies have been based on series of multi-institutional trials since the 1970s. Recent understanding of the importance of biological factors has led to the classification of MB into four distinct molecular groups (Wingless (WNT), Sonic Hedgehog (SHH), Group 3, and Group 4) and 13 subgroups (WNT, four SHH subtypes, and eight Group 3/4 subgroups) with different clinical and prognostic profiles, on which therapeutic stratification is now based. Management of MB in adults is largely based on principles of managing children, modified according to differing clinical and toxicity profiles. Atypical teratoid/rhabdoid tumor (AT/RT) carries mutations in the SMARCB1 gene in most cases, with many now associated with an improved prospect of long-term survival. Through molecular analysis, it is now understood that what was previously referred to as CNS-PNET (primitive neuroectodermal tumor) comprises a morphologically heterogeneous group of tumors (e.g., some are high-grade gliomas), while distinct rare molecular tumor types have emerged after accounting for these, including embryonal tumor with multilayered rosettes (ETMR), CNS neuroblastoma, FOXR2-activated, and CNS tumor with BCOR internal tandem duplication. A significant proportion of patients, mainly with AT/RT and SHH-activated medulloblastoma, has an underlying hereditary cancer predisposition syndrome.

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