A multidisciplinary approach to the successful management of Gorlin syndrome

Abstract

Gorlin–Goltz syndrome (GGS) is a rare genetic syndrome with variable expressivity and autosomal dominant inheritance. The major features of GGS include numerous basal cell carcinomas (BCCs), keratocysts of the jaw, palmar/plantar pits and calcification of the falx cerebri. Authors report the case of a 51-year-old male with a 19-year history of GGS and multiple BCCs of the head and neck. He presented with a large ulcerating lesion on the right side of his face involving cutaneous, subcutaneous and muscular tissues of the temporal and orbital region. Additionally, magnetic resonance imaging revealed involvement of the right zygomatic bone, infratemporal fossa and mandible. This case is notable in that BCC invasion of the facial bones is rare. Extensive resection and reconstruction with a latissimus dorsi microvascular free muscle flap was performed. The success of this challenging case exemplifies the need for a multidisciplinary team that included dermatology, plastic surgery, oculoplastics and otolaryngology.

Oral and Maxillo-Facial Manifestations of Systemic Diseases: An Overview

“…Patients may variably display multiple basal cell carcinomas (also of the head and neck skin), medulloblastomas, frontal ridges, coarse facial features, facial milia, skeletal abnormalities (bisecting ribs and wedge-shaped vertebrae), intellectual disability, heart and ovarian fibromas, palmar and/or plantar pits hyperkeratosis [ 163 , 164 ]. As for head and neck signs/symptoms, patients may be affected by ectopic calcification (especially of the cerebral falx), eye abnormality, lip or palate cleft, single or multiple odontogenic keratocysts of the jaw which are frequently the early sign of the disease and often occasionally detected in paediatric patients [ 165 , 166 , 167 ].…”

Skin Cancer Associated Genodermatoses: A Literature Review

“…Management. Treatment of patients with multiple BCCs should be performed in a multidisciplinary approach, including dermatology, plastic surgery, ophthalmology and, in some cases, dentistry and otolaryngology (13). Since patients may develop multiple BCCs, the treatment should primarily include nonsurgical methods, and, for patients with multiple or aggressive BCCs, treat ment with a hedgehog inhibitor might be indicated (6).…”