Abstract
Background and objectives: Gorlin syndrome is an autosomal dominant, inherited cancer disease with a rare incidence. Diagnostic features are frequently missing in young patients, making the recognition difficult. The presence of two major and one minor criteria or one major and three minor criteria are necessary to establish a diagnosis. The odontogenic keratocyst is frequently the presenting manifestation of this syndrome. This condition may be associated with aggressive basal cell carcinomas and malignant neoplasias in 10% of the patients.
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