Abstract
The reported clinical manifestations of Turcot syndrome were studied to determine whether these corresponded to those of Turcot's original cases. Among the patients with well-documented colonic lesions, the colonic lesions were classified into three groups. First, there was a main group in which colonic lesions had the following characteristics that coincided with those of Turcot's original cases: 1) a low number of polyps (20–100), 2) large polyps over 3 cm in diameter, and 3) complication by colonic cancer during the second or third decades. In the second group, the patients had too few polyps to be diagnosed as polyposis. The third group included patients with numerous colonic polyps similar to those of familial polyposis coli. The recognition of these characteristics of colonic lesions may lead to early detection of glioma in the asymptomatic period.
Similar content being viewed by others
References
Lewis JH, Ginsberg AL, Toomey KE. Turcot's syndrome: evidence for autosomal dominant inheritance. Cancer 1983;51:524–8.
Smith WG, Kern BB. The nature of the mutation in familial multiple polyposis: papillary carcinoma of the thyroid, brain tumors, and familial multiple polyposis. Dis Colon Rectum 1973;16:264–71.
Krockowicz P. The Turcot syndrome. Acta Chir Scand 1979;145:113–5.
McKusick VA. Genetic factors in intestinal polyposis. JAMA 1962;182:271–7.
Baughman FA Jr, List CF, Williams JR, Muldoon JP, Segarra JM, Volkel JS. The glioma-polyposis syndrome. N Engl J Med 1969;281:1345–6.
Itoh H, Ohsato K, Yao T, Iida M, Watanabe H. Turcot's syndrome and its mode of inheritance. Gut 1979;20:414–9.
Cohen SB. Familial polyposis coli and its extracolonic manifestations. J Med Genet 1982;19:193–203.
Cohen SB. Medical aspects of hereditary intestinal polyposis. Dis Colon Rectum 1983;26:401–3.
Turcot J, Després J-P, St Pierre F Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Colon Rectum 1959;2:465–8.
Schröder S, Moehrs D, Weltzien J, Winkler R, Otto HF. The Turcot syndrome: report of an additional case and review of the literature. Dis Colon Rectum 1983;26:533–8.
Scapa E, Umlas J, Loewenstein MS, Zamcheck N. Nonfamilial Turcot's syndrome associated with Crohn's disease and duodenal ulcer in one kindred. Am J Gastroenterol 1983;78:411–2.
Bussey HJR. Familial polyposis coli. Family studies, histopathology, differential diagnosis, and results of treatment. Baltimore: Johns Hopkins University Press, 1975.
Utsunomiya J, Iwama T, Adenomatosis coli in Japan. In: Winawer S, Schottenfeld D, Sherlock P, eds. Colorectal cancer: prevention, epidemiology and screening. New York. Raven Press, 1980:83–95.
Todd DW, Christoferson LA, Leech RW, Rudolf L. A family affected with intestinal polyposis and glioma. Ann Neurol 1981;10:390–2.
Kawanami K, Ohno M, Matsuura K, et al. Turcot's syndrome: report of an autopsy case. Stomach Intestine 1976;11:1075–82.
Michels VV, Stevens JC. Basal cell carcinoma in a patient with intestinal polyposis. Clin Genet 1982;22:80–2.
Nuyts MMJ-P, Leduc M, Ryckewaert Ph, Debruxelles P, Gaudier B. Le syndrome glioma-polypose: discussion nosologique. Bordeaux Médical 1973;6:1601.
Latrive J-P, Chaillet M-P, Veyssier P, Vorhauer W. Syndrome de Turcot. Gastroenterol Clin Biol 1983;7:96–7.
André JM, Picard L, Barrucand D, Kissel P. Les manifestations neurologiques des polypose digestives héréditaires. Rev Neurol 1973;129:325–38.
Kamiya J, Muto T, Morioka K, et al. Turcot syndrome, report of a case. Stomach Intestine 1982;17:913–7.
Warde PJ, Canto AL, Cavallante F, Habr-Gama A. Tumor maligno do sistema nervoso central associado a polipose do cólon (Sindrome de Turcot): appresentacáo de um caso. Arq Gastroenterol 1976;13:119–23.
Crail HW. Multiple primary malignancies arising in the rectum, brain, and thyroid. US Naval Med Bull 1949;49:123–8.
Hamoudi AB, Ertel I, Newton WA Jr, Reiner CB, Clatworthy HW Jr. Multiple neoplasms in an adolescent child associated with IgA deficiency. Cancer 1974;33:1134–44.
Peserico L, Merli GA, Cortesi N, Canossi GC, Crisi G. The Turcot syndrome: case report. Ital J Neurol Sci 1981;4:391–4.
Everson R, Fraumeni JF Jr. Familial glioblastoma with hepatic focal nodular hyperplasia. Cancer 1976;38:310–3.
Franca LCM, Sanvito WL. Tumor maligno do sistema nervoso central associado a polipose do cólon com degeneracao maligna. Arq Neuro-Psiquiat (Sao Paulo) 1969;27:67–72.
Yaffee HS. Gastric polyposis and soft tissue tumors. Arch Dermatol 1966;89:806–8.
Rothman D, Su CP, Kendall A. Dilemma in a case of Turcot's (glioma-polyposis) syndrome: report of a case. Dis Colon Rectum 1975;18:514–5.
Camiel MR, Mulé JE, Alexander LL, Benninghoff DL. Association of thyroid carcinoma with Gardner's syndrome in siblings. N Engl J Med 1968;278:1056–8.
Capps WF Jr, Lewis MI, Gazzaniga DA. Carcinoma of the colon, ampulla of Vater and urinary bladder associated with familial multiple polyposis: a case report. Dis Colon Rectum 1968;11:298–305.
Parks TG, Bussey HJR, Lockhart-Mummery HE. Familial polyposis coli associated with extracolonic abnormalities. Gut 1970; 11:323–9.
Binder MK, Zablen MA, Fleischer DE, et al. Colon polyps, sebaceous cysts, gastric polyps, and malignant brain tumor in a family. Dig Dis 1978;23:460–6.
Author information
Authors and Affiliations
About this article
Cite this article
Itoh, H., Ohsato, K. Turcot syndrome and its characteristic colonic manifestations. Dis Colon Rectum 28, 399–402 (1985). https://doi.org/10.1007/BF02560221
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF02560221