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

Br J Dermatol. 1985 Sep;113(3):295-301. doi: 10.1111/j.1365-2133.1985.tb02081.x.


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.

MeSH terms

  • 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