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Turcot syndrome

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Description

Lynch syndrome is characterized by an increased risk for colorectal cancer (CRC) and cancers of the endometrium, ovary, stomach, small bowel, urinary tract, biliary tract, brain (usually glioblastoma), skin (sebaceous adenomas, sebaceous carcinomas, and keratoacanthomas), pancreas, and prostate. Cancer risks and age of onset vary depending on the associated gene. Several other cancer types have been reported to occur in individuals with Lynch syndrome (e.g., breast, sarcomas, adrenocortical carcinoma). However, the data are not sufficient to demonstrate that the risk of developing these cancers is increased in individuals with Lynch syndrome.

GeneReviews

  • Mode of Inheritance

  • Autosomal recessive inheritance

VARIANTS

168

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Genes

External Links

  • OMIM

    276300

  • Orphanet

    252202

  • HPO
  • Medgen

    C5399763

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