Gorlin syndrome

Definition

Gorlin syndrome, also known as naevoid basal cell carcinoma syndrome (NBCCS), is a hereditary condition characterised by a wide range of developmental abnormalities (odontogenic keratocysts of the jaws, hyperkeratosis of palms and soles, skeletal abnormalities, intracranial ectopic calcifications, and facial dysmorphism) and a predisposition to develop malignant neoplasms (such as multiple basal cell carcinomas or medulloblastoma), and benign neoplasms in the jaw, heart, or ovaries.

Inclusions

• Naevoid basal cell carcinoma syndrome

Index Terms