title: Retrospective study of childhood ganglioneuroma.
abstract: OBJECTIVE
  To review a historical cohort of childhood ganglioneuroma (GN), the benign representative of the peripheral neuroblastic tumor (PNT) family.
  
  
  METHODS
  Of 2,286 PNTs enrolled between 1979 and 2005, 146 (6.4%) were registered as GN. Histological revision was carried out on 76 tumors. Diagnosis was confirmed in 45, while 27 were reclassified as ganglioneuroblastoma intermixed (GNBI) and four were reclassified as other PNT subtypes.
  
  
  RESULTS
  GNs differed from other PNTs for sex, age, tumor site, stage, tumor markers, and scintigraphic results. Characteristics of 76 reviewed and 70 nonreviewed patients were comparable. Reviewed GN and GNBI patients were comparable except for homovanillic acid excretion, metaiodobenzylguanidine scintigraphy, and DNA content. Seven patients were only biopsied and 139 underwent surgery. Twenty-two patients suffered surgery-related complications, of which two were fatal and seven were severe. Radical tumor resection and surgery-related complication rates were comparable for GN, GNBI, and nonreviewed instances. Six patients developed tumor progression but survived. Two patients developed a late malignancy but survived. None of the 146 patients received chemotherapy. Of 146 patients, two died of surgery-related complications and 144 survived.
  
  
  CONCLUSIONS
  Diagnosis was changed to GNBI for approximately one third of 76 reviewed tumors. Patients with confirmed GN, reclassified as GNBI, and nonreviewed histology presented with comparable clinical, biochemical, and biologic features. Surgical results, complication rate, number of progressions, and outcome were similar for the three groups. Surgery was associated with significant risk of complications. Survival was not influenced by extent of tumor resection. Aggressive surgical approach should not be recommended for childhood GN and GNBI.
authors: Department of Pediatric Hematology-Oncology, Giannina Gaslini Children Hospital, Largo Gerolamo Gaslini 5, 16148 Genova, Italy. brunodebernardi@ospedale-gaslini.ge.it
authors: Bruno
authors: B
authors: De Bernardi
authors: Claudio
authors: C
authors: Gambini
authors: Riccardo
authors: R
authors: Haupt
authors: Claudio
authors: C
authors: Granata
authors: Antonino
authors: A
authors: Rizzo
authors: Massimo
authors: M
authors: Conte
authors: Gian Paolo
authors: GP
authors: Tonini
authors: Maurizio
authors: M
authors: Bianchi
authors: Maria
authors: M
authors: Giuliano
authors: Roberto
authors: R
authors: Luksch
authors: Arcangelo
authors: A
authors: Prete
authors: Elisabetta
authors: E
authors: Viscardi
authors: Alberto
authors: A
authors: Garaventa
authors: Angela Rita
authors: AR
authors: Sementa
authors: Paolo
authors: P
authors: Bruzzi
authors: Paola
authors: P
authors: Angelini
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005729:Ganglioneuroma / Q000145:classification / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies