Nevoid basal cell carcinoma syndrome (NBCCS), or Gorlin Syndrome, is a neurocutaneous disorder caused by mutations in the PTCH1 or, less commonly, SUFU genes. Mutations these genes lead to malfunction of sonic hedgehog pathway, causing unregulated proliferation and differentiation. As pathway affects hair follicle growth development, could proliferation. We present case patient whose discrete t...

OBJECTIVES PTCH, the human homologue of the Drosophila segment polarity gene, patched, has been identified as the gene responsible for nevoid basal cell carcinoma syndrome. The aim of this study was to investigate PTCH gene mutation in Chinese patients with nevoid basal cell carcinoma syndrome. MATERIALS AND METHODS DNA was isolated from both odontogenic keratocyst tissue and peripheral blood...

▼ BENIGN “VIRUS-INDUCED” TUMORS (ORAL SQUAMOUS PAPILLOMAS AND WARTS) ▼ SYNDROMES WITH BENIGN ORAL NEOPLASTIC OR HAMARTOMATOUS COMPONENTS Von Recklinghausen’s Neurofibromatosis Gardner’s Syndrome Peutz-Jeghers Syndrome Nevoid Basal Cell Carcinoma Syndrome Multiple Endocrine Neoplasia Type III (Multiple Mucosal Neuroma Syndrome) Tuberous Sclerosis Acanthosis Nigricans Albright’s Syndrome Paget’s ...

Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder with a predisposition to cancer. Features like basal cell carcinoma, odontogenic keratocysts, calcification of falx cerebri, bifid ribs, pits on palms and soles and hypertelorism are evident. A case of this rare disease seen on a 13 year old female patient is presented here, where multiple odontogeni...

Gorlin Goltz Syndrome, or Nevoid Basal Cell Carcinoma is an autosomal dominant genetic disease characterized especially by the manifestation of basal cell carcinomas in skin and keratocysts jaws. Additionally, patients may present with several other manifestations, such as bifid ribs, hypertelorism calcification falx cerebri. The diagnosis has physicians dentists protagonists investigation invo...

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare, autosomal-dominant, cancer-predisposing, multisystem disorder. The clinical manifestations of NBCCS include multiple basal cell carcinomas (BCCs), odontogenic keratocysts, palmar or plantar pits, and calcification of the falx cerebri. We present a case of an 11-year-old boy with Fitzpatrick skin type V who presented with multiple facial le...