Turcot syndrome

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 Hereditary nonpolyposis colorectal cancer and brain tumors like medulloblastoma, malignant glioma. It was first reported by Canadian surgeon Jacques Turcot (1914- ) "et al" in 1959 and hence carries the first author's name. [cite journal |author=Turcot J, Després JP, St. Pierre F |title=Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases |journal=Dis. Colon Rectum |volume=2 |issue= |pages=465–8 |year=1959 |pmid=13839882 |doi= |url=]

The genetic basis of Turcot syndrome is uncertain.The gene most likely to be involved is APC gene in chromosome 5q. [cite book |author=Jameson JN, Kasper DL, Harrison TR, Braunwald E, Fauci AS, Hauser SL, Longo DL. |title=Harrison's principles of internal medicine |publisher=McGraw-Hill Medical Publishing Division |location=New York |year=2005 |edition=16th ed. |pages=2453 |isbn=0-07-140235-7 |oclc= |doi= |accessdate= |url=] However, the syndrome has been claimed to be linked to various mutations in a number of genes. For example, the mismatch repair genes MLH1 [OMIM|120436|MutL, E. COLI, HOMOLOG OF, 1; MLH1] or PMS2 [OMIM|600259|POSTMEIOTIC SEGREGATION INCREASED, S. CEREVISIAE, 2; PMS2] .

ynonyms

* Brain tumor-polyposis syndrome
* Glioma-polyposis syndrome

ee also

* Gardner syndrome is an association of hereditary intestinal polyps and osteomas.

References

External links

*
*RareDiseases|420|Turcot syndrome; CNS tumors with Familial polyposis of the colon


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  • 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… …   Medical dictionary

  • 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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