Síndrome de Gorlin-Goltz: manejo del carcinoma basocelular facial

Abstract

Introduction/objectiveGorlin Goltz syndrome (GGS) is an autosomal dominant inherited disorder that mainly predisposes to the proliferation of tumors, such as basal cell carcinomas and jaw keratocysts. GGS is caused by the Patched gene mutation on chromosome 9. Basal cell carcinomas in patients with GGS usually present as multiple tumors, with polymorphic clinical features, a non-gender predilection, sometimes occurring in the early stages of life, and even affecting areas not exposed to sunlight. The clinical behavior may vary, and sometimes can be very aggressive, especially in the face. In order to study the behavior of basal cell carcinomas in GGS patients, a study was performed on the patients who met criteria for the disease and were treated in our hospital in the period between 2001 and 2011. Material and methodsThe study included 11 patients with clinical and/or genetic diagnosis of GGS. The patients were studied according sex and age, clinical aspects, histological features, surgical treatment provided, presence of recurrence, and follow-up. ResultsBasal cell carcinomas were seen on the face in 36% of the patients. The number of tumors per patient ranged between 9 and 21. The preferred treatment was surgical excision, although all patients developed new lesions and recurrences which required several procedures. The histological study revealed a contact or proximity of the tumor to surgical margins in 28% of lesions. ConclusionsThere is insufficient evidence in the literature to determine the treatment of choice among the different methods available for the management of the basal cell carcinoma in GGS. A preventive approach is necessary to avoid sunlight exposure.