Investigation of the location of atypical teratoid/rhabdoid tumor

Childs Nerv Syst. 2015 Aug;31(8):1305-11. doi: 10.1007/s00381-015-2739-x. Epub 2015 May 8.

Abstract

Introduction: The location of a brain tumor is a fundamental characteristic, because various brain tumors develop in relatively specific locations. An atypical teratoid/rhabdoid tumor (AT/RT) is a highly age-specific tumor that occurs in infants and young children. However, AT/RTs develop in a variety of locations in the brain. This study aimed at uncovering the tumor location pattern of AT/RTs to enhance diagnoses.

Material and methods: Neuroimages from 27 patients with a pathologically proven AT/RT were reviewed, and the specific tumor locations were described and categorized. The association of imaging characteristics and tumor location was analyzed.

Results: The posterior fossa was the most frequent locations accounting for 19 patients (70%), followed by the diencephalon (four patients; 15%), cerebrum (three patients; 11%), and midbrain (one patient; 4%). In the posterior fossa, the superior medullary velum (SMV) and cerebellopontine angle (CPA) areas were the most common sites (eight patients each) and three patients had a tumor in the inferior medullary velum (IMV) region. AT/RTs in the SMV area had a significantly higher chance of no/minimal enhancement compared with tumors in other locations (P = 0.001) and a lower likelihood of leptomeningeal tumor seeding at presentation (P = 0.053).

Conclusion: The location spectrum of AT/RT follows a specific pattern, and some of the locations are linked with intriguing clinical characteristics. This information may not only help make correct preoperative diagnosis but also occasionally aid in postoperative pathological diagnosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brain / pathology*
  • Brain Neoplasms / diagnosis*
  • Child
  • Child, Preschool
  • Chromosomal Proteins, Non-Histone / genetics
  • DNA-Binding Proteins / genetics
  • Female
  • Gadolinium / pharmacokinetics
  • Humans
  • Infant
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Mutation / genetics
  • Retrospective Studies
  • Rhabdoid Tumor / diagnosis*
  • SMARCB1 Protein
  • Spinal Cord / pathology*
  • Teratoma / diagnosis*
  • Transcription Factors / genetics

Substances

  • Chromosomal Proteins, Non-Histone
  • DNA-Binding Proteins
  • SMARCB1 Protein
  • SMARCB1 protein, human
  • Transcription Factors
  • Gadolinium