What is Muir Torre Syndrome
Skin / mucous membrane - symptoms:Muir-Torre syndrome, first described in 1967 and 1968, is a rare autosomal dominant disorder. It is characterized by the occurrence of multiple sebum tumors (adenomas, epitheliomas, carcinomas) with or without keratoacanthomas (i.e. occurrence simultaneously or sequentially) with at least one visceral malignancy without a predisposing factor (upper gastrointestinal tract, endometrium, colorectal and urological malignancies). The peak of manifestation of malignancies is on average 53 years (min. 23 to max. 89 years).
Sebaceous adenomas: Lobed sebum glands of various sizes, which are incompletely differentiated. The center of the peripheral, mature sebum glands is filled with basal cells (characteristic cytoplasmic vacuoles). In adults they appear as yellow papules and nodules, in the sporadic form they are mostly localized on the head (face, scalp, eyelids).
Sebaceous gland carcinomas: Localization mostly on the eyelids, starting from the Maibom's glands or Zeiss' glands; often confused with chalazion, chronic blepharitis, or carbuncles; appear as yellowish, firm nodules with a tendency to ulcerate; Infiltration of orbital adipose tissue; extraorbital carcinomas are prone to metastasis
Keratoacanthoma: benign epithelial skin tumor, in older people (> 65a) especially in sun-exposed areas (hands, face); hemispherical-knot-shaped with a central horn plug, reach a size of 1 to 3 centimeters in a few weeks, often spontaneous regression after 2 to 6 months.
Seboacanthoma: shows features of sebum adenoma and keratoacanthoma
56% of skin lesions appear after the diagnosis of the first malignancy; in 22% of cases, skin tumors are the first sign of the disease.
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Belgium (2), France (1), Ireland (2) [all general cancer support groups!]
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