Muir-Torre syndrome

A.Prof Frank Gaillard et al.

Muir-Torre syndrome is a rare variant of hereditary nonpolyposis colorectal cancer (HNPCC),  characterised by sebaceous neoplasms (e.g adenoma, epithelioma or carcinoma) and keratoacanthocytomas as well as at least one internal malignancy:

  • gastrointestinal malignancies (most common of internal malignancies 3): especially right-sided colorectal carcinoma (CRC)
  • urogenital malignancies (second most common)
  • breast cancer (3rd most common)
  • haematological malignancies 3

It has a male to female ratio of approximately 2:1 

Genetics

It has an autosomal dominant inheritance with variable penetrance.

It was first described by E.G Muir in 1967 and  D.Torre in 1968.

Share article

Article information

rID: 6865
Synonyms or Alternate Spellings:

Support Radiopaedia and see fewer ads

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.