Stanley R. Hamilton, M.D., Bo Liu, Ph.D., Ramon E. Parsons, M.D., Ph.D., Nickolas Papadopoulos, Ph.D., Jin Jen, Ph.D., Steven M. Powell, M.D., Anne J. Krush, M.S., Theresa Berk, M.S.S.A., Zane Cohen, M.D., Bernard Tetu, M.D., Peter C. Burger, M.D., Patricia A. Wood, M.D., Ph.D., Fowzia Taqi, M.D., Susan V. Booker, B.A., Gloria M. Petersen, Ph.D., G. Johan A. Offerhaus, M.D., Anne C. Tersmette, Ph.D., Francis M. Giardiello, M.D., Bert Vogelstein, M.D., and Kenneth W. Kinzler, Ph.D.
Background Turcot's syndrome is characterized clinically bythe concurrence of a primary brain tumor and multiple colorectaladenomas. We attempted to define the syndrome at the molecularlevel.
Methods Fourteen families with Turcot's syndrome identifiedin two registries and the family originally described by Turcotand colleagues were studied. Germ-line mutations in the adenomatouspolyposis coli (APC) gene characteristic of familial adenomatouspolyposis were evaluated, as well as DNA replication errorsand germ-line mutations in nucleotide mismatch-repair genescharacteristic of hereditary nonpolyposis colorectal cancer.In addition, a formal risk analysis for brain tumors in familialadenomatous polyposis was performed with a registry data base.
Results Genetic abnormalities were identified in 13 of the 14registry families. Germ-line APC mutations were detected in10. The predominant brain tumor in these 10 families was medulloblastoma(11 of 14 patients, or 79 percent), and the relative risk ofcerebellar medulloblastoma in patients with familial adenomatouspolyposis was 92 times that in the general population (95 percentconfidence interval, 29 to 269; P<0.001). In contrast, thetype of brain tumor in the other four families was glioblastomamultiforme. The glioblastomas and colorectal tumors in threeof these families and in the original family studied by Turcothad replication errors characteristic of hereditary nonpolyposiscolorectal cancer. In addition, germ-line mutations in the mismatch-repairgene hMLH1 or hPMS2 were found in two families.
Conclusions The association between brain tumors and multiplecolorectal adenomas can result from two distinct types of germ-linedefects: mutation of the APC gene or mutation of a mismatch-repairgene. Molecular diagnosis may contribute to the appropriatecare of affected patients.
Source Information
From the Division of Gastrointestinal and Liver Pathology, Department of Pathology (S.R.H.); the Oncology Center (S.R.H., B.L., R.E.P., N.P., J.J., S.M.P., S.V.B., B.V., K.W.K.); the Division of Medical Genetics, Department of Medicine (A.J.K.); the Division of Neuropathology, Department of Pathology (P.C.B.); the Department of Pathology (F.T., G.J.A.O.); and the Division of Gastroenterology, Department of Medicine (F.M.G.); School of Medicine, and the Department of Epidemiology, the School of Hygiene and Public Health (G.M.P., A.C.T.), Johns Hopkins University, Baltimore; the Familial Gastrointestinal Cancer Registry, Mount Sinai Hospital, Toronto (T.B., Z.C.); the Laboratoire d'Anatomie Pathologique, Hôtel-Dieu de Québec, Quebec, Canada (B.T.); and the Division of Medical Oncology, Department of Medicine, Stratton Veterans Affairs Medical Center and Albany Medical College, Albany, N.Y. (P.A.W.).
Address reprint requests to Dr. Hamilton at the Department of Pathology, Ross Bldg., Rm. 632, Johns Hopkins University School of Medicine, 720 Rutland Ave., Baltimore, MD 21205-2196.
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