Zespół Muira i Torrego

Kod Orpha: 587Kod OMIM: 158320

Definicja

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.

Dane
Klasyfikacja

Choroba

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

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