isotretinoin and Adenoma--Sweat-Gland

We report on a 63-year-old female patient with Muir-Torre syndrome (MTS). In the course of this disease two carcinomas of the colon, a kerato-acanthoma and multiple sebaceous gland tumours, including four sebaceous carcinomas, appeared. This case is thought to be a hereditary form as one of daughters was also found to have a sebaceous epithelioma. MTS is a mostly autosomal-dominant disease with the association of sebaceous gland tumours and internal carcinomas. As the malignant tumours only show slight aggressiveness the prognosis is quite favourable. Oral isotretinoin therapy was successfully used for the inhibition of sebaceous gland proliferation. A narrower definition is presented and an updated survey of the published cases is given. Furthermore, the histopathologic peculiarities of sebaceous gland tumours, especially of sebaceous gland carcinomas, are discussed and compared to sebaceous gland tumours not connected with MTS. A total number of 100 of the 135 published cases of MTS were included and analysed regarding sebaceous gland tumours and other skin tumours. The data on internal carcinomas were taken from the work of Cohen et al. (1991) and 11 current cases were added.

Topics: Adenocarcinoma; Adenoma, Sweat Gland; Adenomatous Polyposis Coli; Administration, Oral; Carcinoma; Carcinoma, Squamous Cell; Chromosome Aberrations; Chromosome Disorders; Diagnosis, Differential; Facial Neoplasms; Female; Genes, Dominant; Humans; Isotretinoin; Keratoacanthoma; Middle Aged; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Neoplastic Syndromes, Hereditary; Sebaceous Gland Neoplasms; Sweat Glands; Syndrome

Topics: Adenoma, Sweat Gland; Female; Humans; Isotretinoin; Middle Aged; Tretinoin

In treating a patient with a malignant eccrine poroma, in-vitro studies suggested resistance to dactinomycin and doxorubicin. A trial of weekly 5-fluorouracil produced no response. Likewise, a cycle of cisplatinum and cytarabine was associated with continued progression of the disease. Use of 13-cis retinoic acid induced a partial remission that lasted for 8 weeks with minimal toxicity. The mechanism of action is not known, but local tumor necrosis with sparing of the adjacent normal tissue suggests high specificity.

Topics: Adenoma, Sweat Gland; Antineoplastic Agents; Drug Therapy, Combination; Humans; Isotretinoin; Lung Neoplasms; Male; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Sweat Gland Neoplasms; Thigh; Tretinoin