Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: a case report.
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.
McLaughlin
M R
MR
Department of Neurological Surgery, Presbyterian University Hospital, University of Pittsburgh Medical Center, PA 15213-2582, USA.
Gollin
S M
SM
Lese
C M
CM
Albright
A L
AL
D000230:Adenocarcinoma / Q000235:genetics; D011125:Adenomatous Polyposis Coli / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D000293:Adolescent; D001932:Brain Neoplasms / Q000235:genetics; D002528:Cerebellar Neoplasms / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002880:Chromosomes, Human, Pair 11; D002899:Chromosomes, Human, Pair 9; D003110:Colonic Neoplasms / Q000235:genetics; D005260:Female; D005909:Glioblastoma / Q000235:genetics; D006801:Humans; D008279:Magnetic Resonance Imaging; D008527:Medulloblastoma / Q000235:genetics; D010296:Parietal Lobe; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic