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Hereditary nonpolyposis colon cancer

Orpha number ORPHA144
Synonym(s) Familial nonpolyposis colon cancer
Familial nonpolyposis colorectal cancer
HNPCC
Hereditary nonpolyposis colorectal cancer
Lynch syndrome
Prevalence Unknown
Inheritance Autosomal dominant
Age of onset Adult
ICD-10
  • C18
  • C18.0
  • C18.1
  • C18.2
  • C18.3
  • C18.4
  • C18.5
  • C18.6
  • C18.7
  • C18.9
ICD-O -
OMIM
UMLS
  • C1333990
MeSH
  • D003123
MedDRA
  • 10051981

Summary

Hereditary nonpolyposis colon cancer (HNPCC) is a cancer-predisposing condition characterized by the development of colorectal cancer (CRC), endometrial cancer, and various other cancers (such as malignant epithelial tumor of ovary, gastric, biliary tract, small bowel, and urinay tract cancer) that are frequently diagnosed at an early age. Two types of HNPCC have been reported: Muir-Torre syndrome and non-polyposis Turcot syndrome (see these terms).


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Detailed information

Review article
  • IT (2010)
  • EN (2010)
Clinical practice guidelines
  • DE (2013)
  • EN (2013)
  • FR (2012)
Diagnostic criteria
  • EN (1999,pdf)
Guidance for genetic testing
  • EN (2012,pdf)
Article for general public
  • IT (2006)
Clinical genetics review
  • EN (2014)
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