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Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: a case report
M R McLaughlin1, S M Gollin, C M Lese
1Department of Neurological Surgery, Presbyterian University Hospital, University of Pittsburgh Medical Center, PA 15213-2582, USA.
Background:
Turcot syndrome (TS) or the glioma-polyposis syndrome, is a rare, heritable disorder thought by some authors to be a variant of familial adenomatous polyposis (FAP). It is characterized by central nervous system (CNS) neoplasms and gastrointestinal polyposis.
Methods:
We present a case report of a patient who developed a medulloblastoma at age 5 years. Ten years later, she developed adenocarcinoma of the colon. Seven months after resection of this Dukes' C2 adenocarcinoma, she presented with a second primary CNS tumor, a glioblastoma multiforme. The patient's colonic adenocarcinoma and glioblastoma were evaluated histologically and cytogenetically.
Results:
Cytogenetic analysis revealed the presence of chromosomal instability in both tumors. This unusual case of two primary CNS neoplasms in a patient with TS is presented with a review of the literature.
Conclusions:
The implications of the cytogenetic analysis are discussed in conjunction with the present knowledge of the molecular biology of TS.