Muir-Torre syndrome

Muir-Torre syndrome is a rare variant of hereditary nonpolyposis colorectal cancer (HNPCC),  characterized 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 (3^rd most common)
• hematological malignancies ³

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.