title: Undifferentiated sinonasal carcinoma in a patient with nevoid basal cell carcinoma syndrome.
abstract: Nevoid basal cell carcinoma syndrome is an autosomal dominant multisystem disorder characterized by developmental anomalies and occurrence of multiple basal cell carcinomas and other tumors in early childhood. In this article, the authors report a case of a 19-year-old African American male with nevoid basal cell carcinoma syndrome and a history of medulloblastoma at age 2, meningioma at age 14, thyroid follicular adenomas with papillary carcinoma at age 15, and 2 basal cell carcinomas at ages 16 and 18. Recently, he developed sinonasal undifferentiated carcinoma (SNUC). The radiology and pathology of the sinonasal carcinoma are presented in this report. Review of the literature reveals that this is the first case of SNUC occurring in a patient with nevoid basal cell carcinoma syndrome.
authors.affiliation: Department of Otolaryngology, Children's National Medical Center, Washington, DC 20010, USA.
authors.forename: Amy
authors.initials: A
authors.lastname: Sobota
authors.forename: Maria
authors.initials: M
authors.lastname: Pena
authors.forename: Mariarita
authors.initials: M
authors.lastname: Santi
authors.forename: Atif
authors.initials: A
authors.lastname: Ali Ahmed
mesh_terms: D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000150:complications* / Q000473:pathology; D002277:Carcinoma / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009669:Nose Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D010256:Paranasal Sinuses / Q000473:pathology