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Turcot's syndrome

Evidence for linkage to the adenomatous polyposis coli (APC) locus

Departments of Neurology (Dr. Wilhelmsen) and Obstetrics and Gynecology (Dr. Lasser), the Division of Pediatric Neurology (Dr. DeVivo), and the Department of Pediatrics (Dr. Garvin), College of Physicians and Surgeons of Columbia University, New York, NY.

Objective: To investigate the possibility that neuroepithelial tumors in Turcot's syndrome are caused by pleiotropic mutations in the gene for adenomatous polyposis coli (APC), a tumor-suppressor gene implicated in colonic cancer.

Methods: We studied the inheritance patterns of genetic markers for the chromosome 5q21 region in 12 members of a Turcot's syndrome kindred with five affected members. We performed linkage analysis to detect linkage between the disease phenotype and DNA markers.

Results: Marker D5S346, located 30 to 70 kilobases from the APC locus, showed evidence highly suggestive of linkage to the disease phenotype (lod score = 1.92).

Conclusions: The data provide evidence that the tumor-suppressor gene implicated in APC and sporadic colon cancers may also cause malignant neuroepithelial tumors in Turcot's syndrome.

Address correspondence and reprint requests to Dr. Kirk C. Wilhelmsen, Department of Neurology, Columbia University School of Medicine, 620 West 168th Street, New York, NY 10032.

Supported by the Herbert Irving Assistant Professorship, the Klingenstein Fellowship, and the Parkinson's Disease and Myoclonus Foundations (K.C.W.), and by the American Gynecological and Obstetrical Society Foundation Award and the Sloane-Rosen Research Scholarship (D.M.L.).

Received August 5, 1993. Accepted for publication in final form December 22, 1993.

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