The Muir-Torre syndrome: a disease of sebaceous and colonic neoplasms.
The Muir-Torre syndrome of sebaceous neoplasms of the skin, with or without keratoacanthomas, and multiple low-grade visceral malignancies with prolonged survival is a rare disorder. Colonic polyps are frequently present, and the syndrome appears to be familial. We report 2 unrelated patients with the Muir-Torre syndrome. Each case exhibited sebaceous adenomas. Gastrointestinal findings included colonic adenocarcinomas and a tubulovillous adenoma. Although an unusual disease, the Muir-Torre syndrome requires recognition because these patients are at risk for multiple primary malignancies and may have family members also at risk.
Schwartz
R A
RA
Department of Dermatology, New Jersey Medical School, Newark.
Goldberg
D J
DJ
Mahmood
F
F
DeJager
R L
RL
Lambert
W C
WC
Najem
A Z
AZ
Cohen
P J
PJ
D000230:Adenocarcinoma / Q000235:genetics*; D000236:Adenoma / Q000235:genetics* / Q000473:pathology; D000328:Adult; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000235:genetics*; D005153:Facial Neoplasms / Q000235:genetics; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000235:genetics* / Q000473:pathology; D012626:Sebaceous Gland Neoplasms / Q000235:genetics* / Q000473:pathology; D012627:Sebaceous Glands / Q000473:pathology; D013577:Syndrome