title: Squamous cell carcinoma of the maxilla in a patient with basal cell nevus syndrome.
abstract: Basal cell nevus syndrome (BCNS) is a rare autosomal dominant disorder with complete penetrance and variable expressivity. This syndrome is characterized by developmental anomalies, such as odontogenic keratocysts of the jaws, multiple basal cell carcinomas, and skeletal abnormalities. Various neoplasms or hamartomas were observed in association with BCNS.We report on a 45-year-old female patient who presented with a tumefaction of the right maxillary region. After correlating clinical, radiographic, and histological features, diagnosis of BCNS associated with squamous cell carcinoma of the right maxillary sinus was made. A surgical treatment was performed. Because of poor compliance and delayed healing, the postoperative radiotherapy was postponed. Three months after the procedure, the patient has developed symptoms of tumoral activity.This clinical report highlights the importance of early diagnosis of BCNS and strict follow-up to prevent severe complications such as squamous cell carcinoma.
authors.affiliation: From the *Univ Lille Nord de France; †Oral and Maxillofacial Department, Roger Salengro Hospital; ‡INSERM U 1008, Controlled Drug Delivery Systems and Biomaterials; and §Service de Chirurgie Plastique, Reconstructrice et Esthétique, Hôpital Roger-Salengro, Lille; and ∥International Association of Oral and Maxillofacial Medicine, Villeneuve-d'Ascq, France.
authors.forename: Romain
authors.initials: R
authors.lastname: Nicot
authors.forename: Corentin
authors.initials: C
authors.lastname: Denglehem
authors.forename: Ahmad
authors.initials: A
authors.lastname: Qassemyar
authors.forename: Gwénaël
authors.initials: G
authors.lastname: Raoul
authors.forename: Joël
authors.initials: J
authors.lastname: Ferri
mesh_terms: D001478:Basal Cell Nevus Syndrome / Q000473:pathology*; D002294:Carcinoma, Squamous Cell / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008441:Maxillary Neoplasms / Q000473:pathology*; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000473:pathology*; D009807:Odontogenic Cysts / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*