Rare Adenoid Variant of Basal Cell Carcinoma in an African American Child With Nevoid Basal Cell Carcinoma Syndrome: Atypical Nodulocystic Presentation.

Nevoid basal cell carcinoma syndrome (NBCCS) (also known as Gorlin-Goltz syndrome, GGS) is a rare autosomal dominant condition with the classic triad of basal cell carcinoma (BCC), keratocystic odontogenic tumor/s, and skeletal malformations. We describe a 15-year-old African American girl who presented with keratocystic odontogenic tumor/s, calcification of the tentorium, and a new development of an atypical nodulocystic lesion on the chest wall. Both fine needle aspiration and subsequent resection showed a rare adenoid cystic variant of BCC. She had a mutation in PTCH1 c.2887+1G > A with a possibly mosaic variant. Development of BCCs is typically in early adulthood in this syndrome and more frequent in individuals of European ancestry. Acrochordon-like lesions are the most common clinical presentation and nodular, follicular, or infundibulocystic are common histologic variants. This case is a rare presentation of BCC in an African American girl with a clinically nodulocystic lesion and unusual adenoid histologic features. This case also emphasizes the significance of a multidisciplinary approach in treating these complex patients.