[Muir-Torre syndrome. Multiple visceral cancers associated with an isolated sebaceous epithelioma].
One case of Muir-Torre Syndrome (MTS) is reported. It is characterized by an unusual association of an unique cutaneous sebaceous epithelioma and multiple visceral adenocarcinomas. The patient was a 67 year-old white woman, from a family with inherited cancer syndrome, who developed previously four colic and one endometrial carcinomas. The morphologic spectrum of the cutaneous tumors observed in the MTS is represented by kerato-acanthoma and/or sebaceous tumors as in the original case described by Torre. The cutaneous tumors are usually multiple and follow after numerous visceral cancers such as colic, endometrial, urinary, or gastric carcinomas. The visceral cancers are remarkable because of the familial character, the multiplicity of tumors and the low-grade malignancy. Our case highlights the difficulty to make an accurate diagnosis for rare histological variants of cutaneous tumors in the MTS.
Vissuzaine
C
C
Service d'Anatomie Pathologique, Hôpital Bichat, Paris.
Vilotte
J
J
Grossin
M
M
D000230:Adenocarcinoma / Q000473:pathology*; D000368:Aged; D002277:Carcinoma / Q000473:pathology*; D005260:Female; D006801:Humans; D009386:Neoplastic Syndromes, Hereditary; D010375:Pedigree; D012878:Skin Neoplasms / Q000473:pathology*
Syndrome de Muir-Torre. Cancers viscéraux multiples associés à un épithélioma sébacé isolé.