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Multiple cutaneous tumors, including sebaceous neoplasms and visceral malignancies. A familial cancer syndrome that combines at least one sebaceous neoplasm (sebaceous adenoma, sebaceous epithelioma, or sebaceous carcinoma) and at least one visceral malignancy (usually gastrointestinal or genitourinary carcinomas).

Cutaneous Sebaceous Neoplasms and Keratoacanthomas, Multiple with Gastrointestinal and Other Carcinomas; Lynch Cancer Family Syndrome II.

A subgroup of this syndrome is part of the Lynch cancer family syndrome II, which has been related to mutation in the MSH2 gene located on 2p. Mutations in the MLH1 gene located in 3p also cause the syndrome.

G.G. Muir and D. Torre first described this syndrome in 1967.

In 1995, 147 cases were described. Patients ranged in age from 37 to 89 years at diagnosis; female-to-male ratio is 2:3.

Autosomal dominant.

Keratoacanthoma can be considered a marker for this syndrome in only two circumstances: (a) if they are multiple and are associated with two or more low-grade visceral malignancies in a patient with a family history of the syndrome, or (b) when a keratoacanthoma displays sebaceous differentiation histologically.

Any sebaceous neoplasm except sebaceous hyperplasia and nevus sebaceous of Jadassohn should signal the possibility of MTS. Because visceral malignancies in MTS are low grade, surgical removal of primary tumor(s), even metastatic ones, may prove valuable and at times appears curative.

Only in relation with the underlying disease leading to the operation.

No specific measures must be undertaken in the anesthetic care of these individuals.

No known pharmacological implications. However, patients receiving radiotherapy or chemotherapy will require special review of the medication used and the potential implications with anesthesia.

Epidermal Nevus Syndrome: Characterized by the presence of linear nevus sebaceus of the midline of the face associated with epilepsy, seizures, and mental retardation. Other clinical features include alopecia, hypoplastic sebaceous glands, verrucous lesions with hyperplastic sebaceous glands developing during adolescence, and benign or malignant tumors developing in the later stage.

Familial Presenile Sebaceous Gland Hyperplasia: Occurs frequently in men older than 50 years. The clinical presentation includes a solitary or several elevated, soft, yellow papules with central umbilication on the face, particularly the forehead. The lesions may spread to the neck and upper part of the thorax.

Esche C, Kruse R, Lamberti C, et al: Muir-Torre syndrome: Clinical features and molecular genetic analysis. Br J Dermatol 136:913, 1997.  [PubMed: 9217825]
Schwartz RA, Torre DP: The Muir-Torre syndrome: A 25-year retrospect. J Am Acad Dermatol 33:90, 1995.  [PubMed: 7601953]

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