GORLIN-SYNDROME - REVIEW OF 11 CASES

Gorlin syndrome is an autosonal dominant disorder with variable penetr ance and expresivity. It is characterized by early onset of multiple b asal cell carcinomas, mandibular keratocysts, pits of the palms and so les, crebral ectopic calcification and several skeletal anomalies. We report a series of eleven patients (eight females and three males) wit h Gorlin syndrome, belonging to nihe different families, which have be en diagnosed in our Dermatology department during the last ten years, The ages at the moment of diagnosis ranged from 8 to 73 years. Ovarian fibromas were demonstrated in five cases and a frontal parasaggital m eningioma in one case. Gorlin syndrome has been recently linked to a p utative tumor suppressor gene which has been mapped to 9(922.3-931), F urther research on the function of the protein encoded by this gene ma y provide additional insight on the mechanisms leading to oncogeneis i n sporadic and hereditary basal cell carcinomas and other tumors.