title: Semiquantitative mIBG scoring as a prognostic indicator in patients with stage 4 neuroblastoma: a report from the Children's oncology group.
abstract: Radiolabeled metaiodobenzylguanidine (mIBG) is a highly sensitive and specific marker for detecting neuroblastoma. A semiquantitative mIBG score (Curie score [CS]) was assessed for utility as a prognostic indicator for a cohort of patients with high-risk metastatic disease.
  
  
  METHODS
  mIBG scans from 280 patients with mIBG-avid, stage 4 neuroblastoma enrolled on the Children's Oncology Group (COG) protocol A3973 were evaluated at diagnosis (n = 280), after induction chemotherapy (n = 237), and after an autologous stem cell transplantation (n = 178). Individual mIBG scans were evaluated at 10 different anatomic regions, with the scoring of each site (0-3) based on the extent of disease at that anatomic region.
  
  
  RESULTS
  There was no correlation between CS at diagnosis and subsequent treatment outcome. Patients with a CS > 2 after induction therapy had a significantly worse event-free survival (EFS) than those with scores ≤ 2 (3-y EFS: 15.4% ± 5.3% vs. 44.9% ± 3.9%, respectively; P < 0.001). A postinduction CS > 2 identified a cohort of patients at greater risk for an event, independent of other known neuroblastoma factors, including age, MYCN status, ploidy, mitosis-karyorrhexis index, and histologic grade. For MYCN-amplified tumors, the presence (CS > 0) versus absence (CS = 0) of residual mIBG avidity after induction was associated with a significantly worse outcome (3-y EFS: 11.8% ± 7.8% vs. 49.6% ± 7.7%, respectively; P = 0.003). After transplantation, patients with a CS > 0 had an EFS inferior to that of patients with a CS of 0 (3-y EFS: 28.9% ± 6.8% vs. 49.3% ± 4.9%, respectively [n = 133]; P = 0.009).
  
  
  CONCLUSIONS
  Curie scoring carries prognostic significance in the management of patients with high-risk neuroblastoma. In particular, patients with CSs > 2 after induction have extremely poor outcomes and should be considered for alternative therapeutic strategies.
authors: Department of Pediatrics, University of Michigan Medical Center, Ann Arbor, MI 48109, USA. gyanik@umich.edu
authors: Gregory A
authors: GA
authors: Yanik
authors: Marguerite T
authors: MT
authors: Parisi
authors: Barry L
authors: BL
authors: Shulkin
authors: Arlene
authors: A
authors: Naranjo
authors: Susan G
authors: SG
authors: Kreissman
authors: Wendy B
authors: WB
authors: London
authors: Judith G
authors: JG
authors: Villablanca
authors: John M
authors: JM
authors: Maris
authors: Julie R
authors: JR
authors: Park
authors: Susan L
authors: SL
authors: Cohn
authors: Patrick
authors: P
authors: McGrady
authors: Katherine K
authors: KK
authors: Matthay
mesh_terms: D019797:3-Iodobenzylguanidine; D000906:Antibodies / Q000627:therapeutic use; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D007457:Iodine Radioisotopes; D008297:Male; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D011877:Radionuclide Imaging; D058028:Research Report; D033581:Stem Cell Transplantation
chemical_list: D000906:Antibodies; D007457:Iodine Radioisotopes; D019797:3-Iodobenzylguanidine