Resolution of Odontogenic Keratocysts of the Jaw in Basal Cell Nevus Syndrome With GDC-0449

Resolution of odontogenic keratocysts of the jaw in basal cell nevus syndrome with GDC-0449.

BACKGROUND Odontogenic keratocysts of the jaw are a central feature of basal cell nevus syndrome (BCNS) and arise from the basal cell layer of the surface epithelium. Although they are benign, they tend to be aggressive, with local invasion of bony structures, extensive growth, and potential for substantial disfigurement and speech dysfunction. Complete surgical resection is the current standar...

Basal cell nevus syndrome presenting as a tender mass of the jaw: A case report

Odontogenic keratocysts in nevoid basal cell syndrome (Gorlin’s Syndrome). CT and MR evaluation

Introduction: The nevoid basal cell syndrome (Gorlin’s Syndrome) is a dominant autosomic disorder, characterized by multiple basal cell carcinomas, odontegenic keratocysts, dural calcifications, bone and face malformations, tumors including meduloblastoma and ovaric fibromas, as well as different degrees of mental retardation. Characteristic imagine findings of the Gorlin’s Syndrome are odontog...

The use of vismodegib to shrink keratocystic odontogenic tumors in patients with basal cell nevus syndrome.

IMPORTANCE Keratocystic odontogenic tumors (KCOTs) of the jaw affect more than 65% of patients with basal cell nevus syndrome (BCNS). Surgery frequently causes facial disfigurement and is not always curative. Most BCNS-related and some sporadic KCOTs have malignant activation of the Hedgehog signaling pathway. OBSERVATIONS We examined the effect of vismodegib (an oral Hedgehog pathway inhibit...

Odontogenic keratocysts in a 5-year-old: initial manifestations of nevoid basal cell carcinoma syndrome.

The purpose of this paper is to report the occurrence of odontogenic keratocysts in a young child. Odontogenic keratocysts are one of the principal features of nevoid basal cell carcinoma syndrome. Their occurrence in this syndrome is usually during the second or third decades of life. This report describes the occurrence of odontogenic keratocysts in a 5-year-old, which proved to be the initia...