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
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
G.G. Muir and D. Torre first described this syndrome in
In 1995, 147 cases were described. Patients ranged in
age from 37 to 89 years at diagnosis; female-to-male ratio is 2:3.
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
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
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
Schwartz RA, Torre DP: The Muir-Torre syndrome: A 25-year retrospect. J Am Acad Dermatol