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 

It has an autosomal dominant inheritance with variable penetrance.

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

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Article information

rID: 6865
Synonyms or Alternate Spellings:

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