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A Journal on Dentistry and Maxillofacial Surgery
Minerva Stomatologica 2002 April;51(4):145-50
Gorlin's syndrome. Case report
Addessi G., Del Vecchio A., Maggiore C., Ripari M.
The authors present a case of Gorlin's Syndrome, more satisfactorily defined as nevoid basal cell carcinoma syndrome (NBCS), a rare genetic disorder characterized mainly by multiple basal cell carcinomas and odontogenic keratocysts and other less frequent skeletal, sexual and neurological manifestations. Patient 75 years old, male. Previously treated surgically for multiple cutaneous basal cell carcinomas, comes to our Department to remove a suspected asymptomatic keratocyst. Clinical examination reveals cutaneous alterations of hands and feet (webbed hands and feet), a little progenism and multiple nevi and basal cell carcinomas on the body and the head. The oral cavity is free of alterations or clinical signs. NBCS is probably caused by chromosomal abnormalities of chromosome 5 and 9. The abnormalities seen in the latter are similar to those seen in people exposed for long periods to UV radiation. These abnormalities could determine malignant cutaneous tumors removing anti neoplastic protection. The association with odontogenic keratocysts, however not clearly understood, appears in more than 90% of cases. All the other disorders are less frequent. Juvenile basal cell carcinomas, expecially if associated with odontogenic keratocysts suggest, the hypothesis of NBCS; if confirmed, this diagnosis makes further familial investigations necessary, to diagnose other cases, at the time unknown.