Journal of Neuro-Oncology

, Volume 95, Issue 2, pp 293–298 | Cite as

Anaplastic oligoastrocytoma in Turcot syndrome

  • Joachim Baehring
  • Pei Hui
  • Joseph Piepmeier
  • Serguei I. Bannykh
Case Report


Turcot syndrome (TS), a rare variant of hereditary non-polyposis colorectal cancer (HNPCC), is characterized by familial clustering of cancer of the large bowel, extracolonic body sites and brain. It is caused by germline mutations in genes encoding for components of the DNA mismatch repair system. We report a 72 year old woman with anaplastic oligoastrocytoma in the setting of TS. Careful analysis of tumor DNA is required to exclude the chance occurrence of a brain tumor in HNPCC kindreds and increase our understanding of the pathogenesis of the disease. Our case adds to the handful of cases published with detailed molecular data previously.


HNPCC Turcot Brain Microsatellite instability Oligoastrocytoma Glioma PTEN 


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Copyright information

© Springer Science+Business Media, LLC. 2009

Authors and Affiliations

  • Joachim Baehring
    • 1
    • 2
  • Pei Hui
    • 3
  • Joseph Piepmeier
    • 2
  • Serguei I. Bannykh
    • 4
  1. 1.Department of NeurologyYale University School of MedicineNew HavenCTUSA
  2. 2.Department of NeurosurgeryYale University School of MedicineNew HavenUSA
  3. 3.Department of PathologyYale University School of MedicineNew HavenUSA
  4. 4.Department of PathologyCedars Sinai Medical CenterLos AngelesUSA

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