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
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Epidemiology
It has a male to female ratio of approximately 2:1
Pathology
Genetics
It has an autosomal dominant inheritance with variable penetrance.
History and etymology
It was first described by EG Muir in 1967 and D Torre in 1968.
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