title: Neuroblastoma in the adult: the Italian experience with 21 patients.
abstract: BACKGROUND
  Neuroblastoma in the adult is rare. No established therapeutic guidelines exist for these patients and the literature on this issue is scant and contradictory.
  
  
  METHODS
  Between 1986 and 2011, 21 adults (18 to 38 y; median, 23) diagnosed with neuroblastoma were referred to our hospital. Three of the 21 were classified as neuroblastoma, not otherwise specified, 13 as neuroblastoma, schwannian stroma-poor, and 5 as ganglioneuroblastoma, nodular. Nine patients had a resectable (stage 1/2) and 6 an unresectable primary tumor (stage 3); 6 had disseminated disease (stage 4).
  
  
  RESULTS
  Of 9 stage 1/2 patients, 6 underwent surgery alone (2 survive, 4 died), 2 received adjuvant chemotherapy (both survive), and 1 received radiation therapy (alive). Four of the 6 stage 3 patients received chemotherapy and died, 1 underwent partial tumor resection only and died, and 1 received radiation therapy after partial tumor resection and is alive. The 6 stage 4 patients received chemotherapy with/without radiotherapy, and all died. Event-free survival at 10 years was 33.3% for stage 1/2, 16.7% for stage 3, and 0% for stage 4 patients. The 10-year overall and event-free survival rates were 39.8% and 19.1%, respectively.
  
  
  CONCLUSIONS
  The outcome of neuroblastoma in adults is poorer than in younger patients at all stages. The clinical course seems modestly influenced by therapy.
authors.affiliation: Departments of *Hematology and Oncology ‡Pathology §Surgery, Istituto Giannina Gaslini †Service of Clinical Epidemiology and Biostatistics ∥Service of Diagnostic Imaging, Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Sorrentino
authors.forename: Anna R
authors.initials: AR
authors.lastname: Gigliotti
authors.forename: Angela R
authors.initials: AR
authors.lastname: Sementa
authors.forename: Vera
authors.initials: V
authors.lastname: Morsellino
authors.forename: Massimo
authors.initials: M
authors.lastname: Conte
authors.forename: Giovanni
authors.initials: G
authors.lastname: Erminio
authors.forename: Piero
authors.initials: P
authors.lastname: Buffa
authors.forename: Claudio
authors.initials: C
authors.lastname: Granata
authors.forename: Katia
authors.initials: K
authors.lastname: Mazzocco
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.forename: Bruno
authors.initials: B
authors.lastname: De Bernardi
mesh_terms: D000293:Adolescent; D000328:Adult; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D018305:Ganglioneuroblastoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D009442:Neurilemmoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D009447:Neuroblastoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D011159:Population Surveillance; D015995:Prevalence; D011379:Prognosis; D055815:Young Adult