title: Ameloblastoma associated with the nevoid basal cell carcinoma (Gorlin) syndrome.
abstract: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder characterized by a wide range of clinical signs and symptoms. The major criteria for the diagnosis are multiple cutaneous basal cell carcinomas, multiple odontogenic keratocysts of the jaw, palmar and plantar pits, and skeletal abnormalities. Here, we report an unusual case of NBCCS in a 68-year-old woman with late onset of clinical signs and symptoms and with an associated ameloblastoma. Only 4 other cases of this unusual association have been reported.
authors.affiliation: Department of Oral Medicine, Infection, and Immunity, Harvard School of Dental Medicine, Boston, MA, USA.
authors.forename: Behnam
authors.initials: B
authors.lastname: Eslami
authors.forename: Carol
authors.initials: C
authors.lastname: Lorente
authors.forename: David
authors.initials: D
authors.lastname: Kieff
authors.forename: Paul A
authors.initials: PA
authors.lastname: Caruso
authors.forename: William C
authors.initials: WC
authors.lastname: Faquin
mesh_terms: D000368:Aged; D000564:Ameloblastoma / Q000209:etiology*; D001478:Basal Cell Nevus Syndrome / Q000150:complications* / Q000235:genetics; D002899:Chromosomes, Human, Pair 9; D005260:Female; D006801:Humans; D007573:Jaw Neoplasms / Q000150:complications* / Q000235:genetics; D008441:Maxillary Neoplasms / Q000209:etiology*; D000072079:Patched Receptors; D000072081:Patched-1 Receptor; D011956:Receptors, Cell Surface / Q000235:genetics
chemical_list: C000606236:PTCH1 protein, human; D000072079:Patched Receptors; D000072081:Patched-1 Receptor; D011956:Receptors, Cell Surface