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Ganglioglioma in a patient with Turcot syndrome. Case report.

Abstract
A 33-year-old woman with Turcot syndrome harbored a brain tumor and colon cancer and had a familial history of this syndrome. On histological examination, the brain tumor was found to have large and diffusely scattered ganglion cells within a diffuse background of astrocytic cells in a fibrillary matrix. The tumor was diagnosed as a ganglioglioma. No germline mutation in the adenomatous polyposis coli gene was detected using a protein truncation assay. These findings indicate that this patient had brain tumor-polyposis syndrome Type 1 of Turcot syndrome. This is the first report of a ganglioglioma related to Turcot syndrome.
AuthorsT Tamiya, S Hamazaki, Y Ono, K Tokunaga, K Matsumoto, T Furuta, T Ohmoto
JournalJournal of neurosurgery (J Neurosurg) Vol. 92 Issue 1 Pg. 170-5 (Jan 2000) ISSN: 0022-3085 [Print] United States
PMID10616098 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)
Topics
  • Adenomatous Polyposis Coli (diagnosis, pathology, surgery)
  • Adult
  • Brain Neoplasms (diagnosis, pathology, surgery)
  • Colorectal Neoplasms, Hereditary Nonpolyposis (diagnosis, pathology, surgery)
  • Female
  • Ganglioglioma (diagnosis, pathology, surgery)
  • Humans
  • Magnetic Resonance Imaging

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