CLINICAL REPORTS   

Chirurgia 1999 April;12(2):139-42

Copyright © 1999 EDIZIONI MINERVA MEDICA

language: Italian

Multiple Hamartoma Syndrome (MHS)

Corsale I., Agozzino L., Nappo C., Esposito S., Dinacci G., Conte R.

Multiple Hamartoma Syndrome (MHS) is a rare, familiar, genodermatosis, characterized by hamartomatous or neoplastic lesions of several organs. About 70% of the patients shows involvement of the thyroid gland: thyroiditis, lonely or multiple adenomas, goiter and, in the 10% of cases, carcinomas. In the 50% of the MHS we find pathological lesions of the breast: fibrocystic disease, fibroadenomas, ductal papillomas. 28-30% of the patients, can develops a breast carcinoma. Two familiar cases of MHS, one affected by proliferating trichilemmomas, other by fibrocystic disease are presented. A scheme of follow-up with annual breast and thyroid screening, and gastroenterological, urologic and gynecologic every three years is proposed.