Atypical teratoid/rhabdoid tumor evolving from an optic pathway ganglioglioma: case study

Neuro Oncol. 2006 Jan;8(1):79-82. doi: 10.1215/S1522851705000347.

Abstract

We report an atypical teratoid/rhabdoid tumor arising in a ganglioglioma from an 11-year-old male who had been treated over a nine-year period. A combined histologic, immunohistochemical, and molecular genetic analysis confirmed this diagnosis. Molecular genetic studies demonstrated a mutation in exon 9 of the INI1 gene in the tumor, which was not present in the patient's blood. This report is the first to describe progression of a ganglioglioma to atypical teratoid/rhabdoid tumor.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Amino Acid Sequence
  • Base Sequence
  • Brain Neoplasms / genetics
  • Brain Neoplasms / pathology*
  • Child
  • Chromosomal Proteins, Non-Histone
  • Chromosomes, Human, Pair 22 / genetics
  • DNA Mutational Analysis
  • DNA-Binding Proteins / genetics
  • Fatal Outcome
  • Female
  • Frameshift Mutation
  • Ganglioglioma / genetics
  • Ganglioglioma / pathology*
  • Humans
  • Male
  • Neoplasms, Multiple Primary / genetics
  • Neoplasms, Multiple Primary / pathology*
  • Rhabdoid Tumor / genetics
  • Rhabdoid Tumor / pathology*
  • SMARCB1 Protein
  • Teratoma / genetics
  • Teratoma / pathology*
  • Transcription Factors / genetics

Substances

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