Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: a case report.

Abstract

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.

• Authors: M R McLaughlin, S M Gollin, C M Lese, A L Albright
• Journal: Surgical neurology (Surg Neurol) Vol. 49 Issue 3 Pg. 295-301 (Mar 1998) ISSN: 0090-3019 [Print] United States
• PMID: 9508118 (Publication Type: Case Reports, Journal Article)

Topics

• Adenocarcinoma (genetics)
• Adenomatous Polyposis Coli (diagnosis, genetics, therapy)
• Adolescent
• Brain Neoplasms (genetics)
• Cerebellar Neoplasms (genetics)
• Child
• Child, Preschool
• Chromosomes, Human, Pair 11
• Chromosomes, Human, Pair 9
• Colonic Neoplasms (genetics)
• Female
• Glioblastoma (genetics)
• Humans
• Magnetic Resonance Imaging
• Medulloblastoma (genetics)
• Parietal Lobe
• Tomography, X-Ray Computed
• Translocation, Genetic