Muir-Torre syndrome

Orpha code: 587OMIM code: 158320

Definition

A form of hereditary nonpolyposis colon cancer characterized by the development of cutaneous sebaceous neoplasia and at least one visceral malignancy, most frequently gastrointestinal carcinoma. The malignancies are usually multiple, occur at an early age, but tend to be of low-grade and have a relatively low incidence of metastases. Sebaceous tumors are usually multiple, with sebaceous adenomas being the commonest. Multiple keratoacanthomas, usually located on the face or the trunk, have been reported as a feature. Cutaneous tumors may precede or follow the first presentation of internal malignancy, which usually involves the gastrointestinal tract, the breast or the genitourinary tract.

Disease data
Classification

Disease

Synonyms
Multiple keratoacanthoma, Muir-Torre type
Rogowiak kolczystokomórkowy typu Muira i Torrego
ORPHA code
587
OMIM code
158320
ICD10 code
L72.8
ICD11 code
2C31.1

No additional description.

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