title: [Pediatric keratocystic odontogenic tumor and nevoid basal cell carcinoma syndrome. Predictive factors for recurrence and aggressiveness].
abstract: BACKGROUND
  Keratocystic odontogenic tumors (KOT), as complications in Nevoid Basal Cell Carcinoma Syndrome (NBCCS), occur early (before 20 years of age) and are usually more aggressive. The aim of this retrospective study was to determine the clinical, histological, and genetic phenotype, of these lesions and to define predictive features of aggressiveness.
  
  
  METHODS
  We retrospectively studied five patients presenting with one or several KOT with NBCCS. We collected their clinical, radiological, and therapeutic data, rate of recurrence or new localization. Anatomopathological examinations were reviewed systematically. Somatic PTCH, SMO and SMAD 4 sequencing were completed.
  
  
  RESULTS
  The average age at diagnosis was 11.2 years. The average number of KOT was 3.2 most often located in the molar region. All the cysts were enucleated. Anatomopathological examination revealed the presence of satellite cysts and daughter cysts and epithelial expansion in more than 80% of cases. No somatic mutation was observed among KOT.
  
  
  CONCLUSIONS
  KOT develop in the first 10 years, in patients presenting with NBCCS, and recurrence is observed in the second and third decade. KOT are typically aggressive and have a tendency to recur, especially in patients with NBCCS. Anatomopathological examination may be predictive of the lesion's aggressiveness. Understanding the genetic and immunological mechanisms should open the way for new medical treatment.
authors.affiliation: Service de Chirurgie Maxillofaciale et Chirurgie Plastique, Hôpital d'Enfants Armand-Trousseau, AP-HP, 26, avenue du Docteur-Arnold-Netter, 75571 Paris cedex 12, France. natacha.kadlub@trs.aphp.fr
authors.forename: N
authors.initials: N
authors.lastname: Kadlub
authors.forename: M-E
authors.initials: ME
authors.lastname: Gatibelza
authors.forename: N
authors.initials: N
authors.lastname: El Houmami
authors.forename: A
authors.initials: A
authors.lastname: Coulomb-Lhermine
authors.forename: V
authors.initials: V
authors.lastname: Descroix
authors.forename: B
authors.initials: B
authors.lastname: Ruhin-Poncet
authors.forename: N
authors.initials: N
authors.lastname: Soufir
authors.forename: M-P
authors.initials: MP
authors.lastname: Vazquez
authors.forename: A
authors.initials: A
authors.lastname: Berdal
authors.forename: A
authors.initials: A
authors.lastname: Picard
mesh_terms: D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D002648:Child; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D008336:Mandibular Diseases / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D008339:Mandibular Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D009361:Neoplasm Invasiveness; D009807:Odontogenic Cysts / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors