Aggressive basal cell carcinoma of the temporal region in a patient with Gorlin-Goltz syndrome.

Abstract

Gorlin-Goltz syndrome is an autosomal dominant disorder with variable penetration characterized primarily by five major findings: multiple basal cell carcinomas presenting at a young age, pits on the palms and soles, skeletal abnormalities, jaw cysts, and ectopic calcification of the falx cerebri and other structures. When the basal cell carcinomas are located in the head and neck there is a high risk of invasion of deep structures if early and radical treatment is not performed. The authors present a 59-year-old man affected by basal cell carcinoma in the context of Gorlin-Goltz syndrome. Although patients with this syndrome can present aggressive basal cell carcinomas, it is unusual to find them involving the craniofacial bones. In this patient the basal cell carcinoma involved the middle ear, the intrapetrous aspect of the facial nerve, and the dura mater. The reconstruction of a wide three-dimensional defect, in which the brain was exposed, was achieved with local flaps and a free musculocutaneous rectus abdominis flap. Factors affecting reconstruction in the lateral cranial base are discussed.