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At a glance

It is a multiple cutaneous tumors disease that includes sebaceous neoplasms and visceral malignancies. Muir-Torre Syndrome is a familial cancer syndrome that combines simultaneously at least one sebaceous neoplasm (sebaceous adenoma, sebaceous epithelioma, or sebaceous carcinoma) and at least one visceral malignancy (usually gastrointestinal or genitourinary carcinomas).

Synonyms

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

History

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

Classification

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.

Incidence

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

Genetic inheritance

Autosomal dominant.

Diagnosis

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. The diagnosis for Muir-Torre Syndrome and Lynch Syndrome is established on the Amsterdam Criteria that are defined as follows: (1) 3 or more relatives with an HNPCC-associated cancer (ie, colorectal, cancer of the endometrium, small bowel, ureter, or renal pelvis); (2) 2 or more successive generations affected by cancer; and (3) 1 or more persons with cancer is a first-degree relative of the other 2, at least 1 case of colorectal cancer younger than age 50 years, a diagnosis of familial adenomatous polyposis has been excluded, tumors are verified by histologic examination.

Clinical aspects

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.

Precautions before anesthesia

The preoperative evaluation pertains only in relation with the underlying disease and possible consequences (eg, tumor causing vascular compression) leading to the operation.

Anesthetic considerations

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

Pharmacological implications

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

Other conditions to be considered

  • Epidermal Nevus Syndrome: Characterized by the presence of linear nevus sebaceus of the midline ...

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