Muir-Torre syndrome in several members of a family with a variant of the Cancer Family Syndrome.

Abstract

Distinguishing cutaneous signs which are associated with hereditary cancer-prone syndromes are known as cancer-associated genodermatoses. Muir-Torre syndrome (M-T) is characterized by the occurrence of sebaceous hyperplasia, adenoma and carcinoma, basal cell carcinoma with sebaceous differentiation, and/or keratoacanthoma in association with visceral cancer (often multiple), and improved survival. Family studies of M-T have been either wholly lacking or too incomplete to elucidate hereditary aetiology. We describe the cutaneous phenotype of M-T in an extended kindred with a possible variant of the Cancer Family Syndrome. We emphasize the need for more thorough documentation of family histories and cancer association in this cancer-associated genodermatosis in order to clarify hereditary syndrome identification, and to improve cancer control through employment of cutaneous signs as a beacon for highly targeted forms of visceral cancer.

• Authors: H T Lynch, R M Fusaro, L Roberts, G J Voorhees, J F Lynch
• Journal: The British journal of dermatology (Br J Dermatol) Vol. 113 Issue 3 Pg. 295-301 (Sep 1985) ISSN: 0007-0963 [Print] England
• PMID: 4063166 (Publication Type: Journal Article)

Topics

• Adolescent
• Adult
• Aged
• Colonic Neoplasms (genetics)
• Female
• Humans
• Hyperplasia (genetics)
• Keratoacanthoma (genetics)
• Male
• Middle Aged
• Neoplasms, Multiple Primary (genetics)
• Pedigree
• Sebaceous Glands (pathology)
• Skin Diseases (genetics)
• Skin Neoplasms (genetics)
• Syndrome