Turcot syndrome

Turcot syndrome
A rare disease characterized by multiple growths called polyps in the colon (large intestine) together with tumors in the brain. The polyps in the colon tend to become malignant. The brain tumors (gliomas and astrocytomas) in Turcot syndrome are also malignant. There are sometimes also skin abnormalities including cafe-au-lait (coffee-with-milk) spots, multiple lipomas (fatty tumors), and multiple scalp basal cell carcinoma (skin cancers of the scalp). Turcot syndrome is inherited in an autosomal recessive manner with both parents carrying a Turcot gene and a 1 in 4 risk for each of their boys and girls of receiving both parental Turcot genes and suffering from the syndrome. Turcot syndrome is clinically considered a variant of a disease called familial adenomatous polyposis (adenomatous polyposis coli). There is genetic evidence that Turcot syndrome is due to mutations in either the adenomatous polyposis coli (APC) gene or in what are known as the mismatch repair genes MLH1 or PMS2. Turcot, a French Canadian, pronounced his name with a silent terminal "t."
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See under syndrome.

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familial adenomatous polyposis of the colon associated with malignant tumors (gliomas) of the central nervous system.

Medical dictionary. 2011.

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  • Turcot syndrome — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 29793 ICD10 = ICD9 = ICDO = OMIM = 276300 MedlinePlus = eMedicineSubj = ped eMedicineTopic = 828 MeshID = Turcot syndrome is the association between familial adenomatous polyposis or… …   Wikipedia

  • Turcot syndrome — /ˈtɜkoʊ sɪndroʊm/ (say terkoh sindrohm) noun Medicine a rare condition caused by a faulty gene in which cells become abnormal and form polyps, usually in the brain and the bowel lining. Abbrev.: TS Also, Turcot s syndrome. {named after Jacques… …  

  • Syndrome de turcot — Pour les articles homonymes, voir Turcot. Le syndrome de Turcot est une association assez rare : d une polypose recto colique familiale d une tumeur du système nerveux central : astrocytome glioblastome médulloblastome Ce document… …   Wikipédia en Français

  • Turcot — Cette page d’homonymie répertorie les différents sujets et articles partageant un même nom. Personnalité Jean Turcot ( 1652) est un pionnier de la Nouvelle France. Louise Turcot est une actrice québécoise née le 5 octobre 1944. Philippe Turcot… …   Wikipédia en Français

  • Syndrome de Turcot — Pour les articles homonymes, voir Turcot. Le syndrome de Turcot est une association assez rare : d une polypose recto colique familiale d une tumeur du système nerveux central : astrocytome glioblastome médulloblastome …   Wikipédia en Français

  • Syndrome de Lynch — Cancer colorectal héréditaire sans polypose Cancer colorectal héréditaire sans polypose Autre nom Syndrome de Lynch Syndrome de Turcot HNPCC Référence MIM …   Wikipédia en Français

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Mismatch repair cancer syndrome — Classification and external resources OMIM 276300 DiseasesDB 29793 eMedicine …   Wikipedia

  • glioma-polyposis syndrome — Turcot s …   Medical dictionary

  • Síndrome de Turcot — Imagen por resonancia magnética en corte coronal de la cabeza que demuestra la presencia de glioblastoma multiforme en el cerebro, uno de los tipos de tumores del sistema nervioso …   Wikipedia Español

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