title: Treatment of esthesioneuroblastomas.
abstract: OBJECTIVE
  To analyze the clinical features, treatment outcomes, pattern of failures, and course of the disease of a cohort of patients treated for esthesioneuroblastoma (ENB) with craniofacial resection (CFR) at a single institution during a 12-year period.
  
  
  METHODS
  Retrospective analysis of 11 patients with ENB treated with CFR in a tertiary care academic medical center from 1998 to 2009.
  
  
  RESULTS
  Median age at diagnosis was 51 years (range 41-67 years). The most common presenting symptom was nasal obstruction (91%). Four patients (36%) presented with Kadish stage B, six patients (55%) with Kadish stage C, and one patient (9%) with Kadish stage D. The initial treatment was craniofacial resection (CFR) alone for three patients (23%), CFR followed by postoperative radiation therapy (RT) in seven patients (64%), while one patient (9%) received both neoadjuvant and adjuvant RT in addition to surgery. The mean and median follow-up times were 66 and 58 months, respectively (range 23-158 months). Seven patients are currently alive with no evidence of disease (64%), while two patients are alive with disease (18%). Overall survival was 100% at one year postoperatively and 80% five years after the primary treatment. The progression free survival was calculated to 73% at one year and 64% at five years.
  
  
  CONCLUSIONS
  ENB is an uncommon diagnosis with an incidence of 0.037/100,000 persons/year in the catchment area of our institution. Treatment can be challenging, especially with advanced disease. CFR with RT offers good oncologic disease control with minimal morbidity.
authors: Department of neurology, Ostfold hospital trust, 1603 Fredrikstad, Norway. Electronic address: m.steinberger.konig@gmail.com.
authors: M S
authors: MS
authors: König
authors: Department of otorhinolaryngology, Oslo university hospital, Rikshospitalet, 0027 Oslo, Norway. Electronic address: terje.osnes@oslo-universitetssykehus.no.
authors: T
authors: T
authors: Osnes
authors: Department of neurosurgery, Oslo university hospital, Rikshospitalet, 0027 Oslo, Norway. Electronic address: torstein.meling@oslo-universitetssykehus.no.
authors: T R
authors: TR
authors: Meling
mesh_terms: D000328:Adult; D000368:Aged; D018572:Disease-Free Survival; D018304:Esthesioneuroblastoma, Olfactory / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D019635:Neurosurgical Procedures / Q000379:methods*; D009664:Norway; D009669:Nose Neoplasms / Q000601:surgery*; D010255:Paranasal Sinus Neoplasms / Q000601:surgery*; D011182:Postoperative Care; D011446:Prospective Studies; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Complications; Craniotomy; Crâniotomie; Esthesioneuroblastoma; Esthésioneuroblastome; Morbidité chirurgicale; Outcome; Résultat; Surgical morbidity