title: Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed.
abstract: Ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBI) are mature variants of neuroblastic tumors (NT). It is still discussed whether incomplete resection of GN/GNBI impairs the outcome of patients.
  
  
  
  Clinical characteristics and outcome of localized GN/GNBI were retrospectively compared to localized neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) registered in the German neuroblastoma trials between 2000 and 2010.
  
  
  
  Of 808 consecutive localized NT, 162 (20 %) were classified as GN and 55 (7 %) as GNBI. GN/GNBI patients presented more often with stage 1 disease (68 % vs. 37 %, p < 0.001), less frequently with adrenal tumors (31 % vs. 43 %, p = 0.001) and positive mIBG-uptake (34 % vs. 90 %, p < 0.001), and had less often elevated urine catecholamine metabolites (homovanillic acid 39 % vs. 62 %, p < 0.001, vanillylmandelic acid 27 % vs. 64 %, p < 0.001). Median age at diagnosis increased with grade of differentiation (NB/GNBN: 9; GNBI: 61; GN-maturing: 71; GN-mature: 125 months, p < 0.001). Complete tumor resection was achieved at diagnosis in 70 % of 162 GN and 67 % of 55 GNBI, and after 4 to 32 months of observation in 4 GN (2 %) and 5 GNBI (9 %). Eleven patients received chemotherapy without substantial effect. Fifty-five residual tumors (42 GN, 13 GNBI) are currently under observation (median: 44 months). Five patients (3 GN, 2 GNBI) showed local progression; all had tumor residuals > 2 cm. No progression occurred after subtotal resection. Two patients died of treatment, none of tumor progression.
  
  
  
  GN/GNBI account for one quarter of localized NT and differ from immature tumors in their clinical features. Chemotherapy is not effective. Subtotal resection appears to be a sufficient treatment.
  
  
  
  ClinicalTrials.gov identifiers - NB97 (NCT00017225; registered June 6, 2001); NB2004 (NCT00410631; registered December 11, 2006).
authors.affiliation: Department of Pediatric Hematology and Oncology, Children's Hospital, University of Cologne, Cologne, Germany.
authors.forename: Boris
authors.identifier: 0000-0002-1607-0543
authors.initials: B
authors.lastname: Decarolis
authors.affiliation: Department of Pediatric Hematology and Oncology, Children's Hospital, University of Cologne, Cologne, Germany.
authors.forename: Thorsten
authors.initials: T
authors.lastname: Simon
authors.affiliation: Department of Radiology, University of Cologne, Cologne, Germany.
authors.forename: Barbara
authors.initials: B
authors.lastname: Krug
authors.affiliation: Department of Pathology, University of Kiel, Kiel, Germany.
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.affiliation: Department of Pathology, University of Kiel, Kiel, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Vokuhl
authors.affiliation: German Childhood Cancer Registry, University of Mainz, Mainz, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Kaatsch
authors.affiliation: Department of Pediatric Surgery, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Munich, Germany.
authors.forename: Dietrich
authors.initials: D
authors.lastname: von Schweinitz
authors.affiliation: Clinic for Pediatric Hematology and Oncology, Johann Wolfgang Goethe-University, Frankfurt, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Klingebiel
authors.affiliation: Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg-Eppendorf, Germany.
authors.forename: Ingo
authors.initials: I
authors.lastname: Mueller
authors.affiliation: Clinic for Pediatrics, Helios Klinikum Berlin-Buch, Berlin-Buch, Germany.
authors.forename: Lothar
authors.initials: L
authors.lastname: Schweigerer
authors.affiliation: Department of Pediatric Hematology and Oncology, Children's Hospital, University of Cologne, Cologne, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Berthold
authors.affiliation: Department of Pediatric Hematology and Oncology, Children's Hospital, University of Cologne, Cologne, Germany. barbara.hero@uk-koeln.de.
authors.forename: Barbara
authors.initials: B
authors.lastname: Hero
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004358:Drug Therapy / Q000379:methods*; D013507:Endocrine Surgical Procedures / Q000379:methods*; D005260:Female; D018305:Ganglioneuroblastoma / Q000628:therapy*; D005729:Ganglioneuroma / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009367:Neoplasm Staging; D012189:Retrospective Studies; D061665:Time-to-Treatment; D016896:Treatment Outcome
keywords: Ganglioneuroblastoma intermixed; Ganglioneuroma; Residual tumor; Subtotal resection; Surgery; Therapy; Treatment