Poster Presentation Advances in Neuroblastoma Research Congress 2016

Validation of image-defined risk factor (IDRF) assignment in patients with intermediate-risk neuroblastoma: a report from the Children’s Oncology Group study ANBL0531 (#273)

Clare J. Twist 1 , E. Stanton Adkins 2 , Peter Mattei 3 , Sandra L. Wooton-Gorges 4 , Fredric A. Hoffer 5 , Arlene Naranjo 6 , Mary L. Schmidt 7 , Michael D. Hogarty 8 , Meredith S. Irwin 9 , Susan L. Cohn 10 , Julie R. Park 11 , John M. Maris 8
  1. Divison of Pediatric Hematology/Oncology, Stanford University, Palo Alto, CA, USA
  2. South Carolina Cancer Center, Charleston, SC, USA
  3. Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA
  4. Department of Radiology, University of California at Davis, Davis, CA, USA
  5. Quality Assurance Review Center, Lincoln, RI, USA
  6. Children's Oncology Group Statistics and Data Center, University of Florida , Gainsville, FL, USA
  7. Division of Pediatric Hematology/Oncology, University of Illinois, Chicago, IL, USA
  8. Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
  9. Division of Hematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada
  10. Division of Pediatric Hematology/Oncology, University of Chicago, Chicago, IL, USA
  11. Division of Pediatric Hematology/Oncology, Seattle Children's Hospital, Seattle, WA, USA

Background: The International Neuroblastoma Risk Group (INRG) classifier utilizes a staging system based on pretreatment imaging criteria, with the extent of locoregional disease determined by the presence or absence of IDRF. An objective of the Phase III study ANBL0531 was to prospectively validate the prognostic value of the INRG IDRF system and to compare the institutional determination of IDRF with that of a central review committee.

 

Methods: Between 9/2009 and 6/2011, 211 newly diagnosed eligible intermediate risk patients enrolled on ANBL0531 had assessment of primary tumor IDRF performed by their local institution. Risk-stratification and therapy assignment utilized age, INSS stage, INPC, MYCN, and tumor ploidy, but not IDRF status or INRG staging. Therapy reduction was prescribed for tumors without loss of heterozygosity at 1p36 and/or 11q23. Following study closure, blinded central review of initial diagnostic imaging was performed by a panel of pediatric surgeons and radiologists to validate the local institutional assessment of IDRF.

 

Results: The cohort of 211 patients included 12 INSS stage 2A, 16 stage 2B, 83 stage 3, 74 stage 4, and 26 stage 4S neuroblastoma. The 3-year overall survival rate for patients with localized tumors, regardless of tumor biology, was 100%. The accuracy of IDRF assignment by local institutions will be compared to central review assignment and will be reported at the meeting. The potential impact of primary tumor location and number or type of IDRF on choice of initial diagnostic procedures and surgical morbidity will also be presented.

 

Conclusions: These data will inform the process of transition from INSS to INRG staging, including the impact of IDRF on the choice of the initial diagnostic and surgical procedures, the risk of surgical complications, and survival rates for patients with localized tumors, whether defined as INSS stage 2A/2B, 3 or INRG L2.