Odontogenic Keratocysts as First Manifestation of Nevoid Basal Cell Carcinoma Syndrome: Surgical Management and Immunohistochemical Analysis

Nevoid Basal Cell Carcinoma Syndrome (NBCCS), also known as Gorlin syndrome, is a rare autosomal dominant disorder, with no gender predilection. Individuals with NBCCS are commonly diagnosed between 17 and 35 years old and can present multiple basal cell carcinomas scattered throughout the body, presence of recurrent and early-onset odontogenic keratocysts (OKCs) and skeletal abnormalities. This article describes a case of a 13-year-old white boy who referred complaining of facial asymmetry. Extraoral examination revealed volumetric increase displacing the nasal ala from the right side and extended to the zygomatic bone. The intraoral evaluation showed mixed dentition with moderate degree of malocclusion. In addition, bilateral vestibular fornix swelling was observed in the upper canine region. An increase in volume was also detected on the hard palate on the right side. Computed tomography revealed multiple hypodense lesions with cystic appearance. The aspiration was positive, with a yellowish aspirate of serous consistency of all lesions. Given the numerous lesions, it was decided to decompress them for posterior enucleation procedures. In addition to other manifestations, the patient was diagnosed with NBCCS. Although common, the occurrence of OKCs in pediatric patients, especially in multiple lesions, is highly indicative of NBCCS, and its investigation should be considered, even in the absence of other signs of this syndrome. Synchronous decompression was satisfactory and can be used in similar cases of multiple cystic lesions.