Embryonal Tumors of the Central Nervous System: An Update

Surg Pathol Clin. 2020 Jun;13(2):235-247. doi: 10.1016/j.path.2020.01.003. Epub 2020 Apr 7.

Abstract

Embryonal tumors of the central nervous system (CNS) are rare, high-grade neoplasms predominantly affecting the pediatric population. Well-defined embryonal tumors include medulloblastoma, atypical teratoid/rhabdoid tumor, embryonal tumor with multilayered rosettes, C19MC-altered and embryonal tumor with multilayered rosettes, not otherwise specified, pineoblastoma, pituitary blastoma, CNS neuroblastoma, and ganglioneuroblastoma. Although their prognosis is nearly uniformly poor, the rapidly evolving understanding of their molecular biology contributes to diagnosis, prognosis, treatment, and clinical trial participation. Knowledge of current tumor stratification and diagnostic techniques will help pathologists guide care and preserve tissue for necessary or desired additional testing.

Keywords: Atypical teratoid rhabdoid tumor; CNS embryonal tumors; Embryonal tumor with multilayered rosettes; High-grade neuroepithelial tumor; Medulloblastoma.

Publication types

  • Review

MeSH terms

  • Central Nervous System Neoplasms / diagnosis*
  • Central Nervous System Neoplasms / pathology
  • Central Nervous System Neoplasms / therapy
  • Cerebellar Neoplasms / diagnosis
  • Cerebellar Neoplasms / pathology
  • Cerebellar Neoplasms / therapy
  • Humans
  • Medulloblastoma / diagnosis
  • Medulloblastoma / pathology
  • Medulloblastoma / therapy
  • Neoplasms, Germ Cell and Embryonal / diagnosis*
  • Neoplasms, Germ Cell and Embryonal / pathology
  • Neoplasms, Germ Cell and Embryonal / therapy
  • Prognosis
  • Rhabdoid Tumor / diagnosis
  • Rhabdoid Tumor / pathology
  • Rhabdoid Tumor / therapy
  • Teratoma / diagnosis
  • Teratoma / pathology
  • Teratoma / therapy

Supplementary concepts

  • Teratoid Tumor, Atypical