Article

The International Neuroblastoma Risk Group (INRG) staging system: an INRG Task Force report.

Section for Paediatric Surgery, Division of Surgery, Rikshospitalet University Hospital, NO-0027 Oslo, Norway.
Journal of Clinical Oncology (Impact Factor: 17.88). 01/2009; 27(2):298-303. DOI: 10.1200/JCO.2008.16.6876
Source: PubMed

ABSTRACT The International Neuroblastoma Risk Group (INRG) classification system was developed to establish a consensus approach for pretreatment risk stratification. Because the International Neuroblastoma Staging System (INSS) is a postsurgical staging system, a new clinical staging system was required for the INRG pretreatment risk classification system.
To stage patients before any treatment, the INRG Task Force, consisting of neuroblastoma experts from Australia/New Zealand, China, Europe, Japan, and North America, developed a new INRG staging system (INRGSS) based on clinical criteria and image-defined risk factors (IDRFs). To investigate the impact of IDRFs on outcome, survival analyses were performed on 661 European patients with INSS stages 1, 2, or 3 disease for whom IDRFs were known.
In the INGRSS, locoregional tumors are staged L1 or L2 based on the absence or presence of one or more of 20 IDRFs, respectively. Metastatic tumors are defined as stage M, except for stage MS, in which metastases are confined to the skin, liver, and/or bone marrow in children younger than 18 months of age. Within the 661-patient cohort, IDRFs were present (ie, stage L2) in 21% of patients with stage 1, 45% of patients with stage 2, and 94% of patients with stage 3 disease. Patients with INRGSS stage L2 disease had significantly lower 5-year event-free survival than those with INRGSS stage L1 disease (78% +/- 4% v 90% +/- 3%; P = .0010).
Use of the new staging (INRGSS) and risk classification (INRG) of neuroblastoma will greatly facilitate the comparison of risk-based clinical trials conducted in different regions of the world.

1 Bookmark
 · 
438 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: The European multicenter study LNESG1 was designed to evaluate the safety and efficacy of surgical treatment alone in patients with localised neuroblastoma. In a retrospective, observational study we examined the impact of image-defined risk factors (IDRF) on operative complications and survival (EFS and OS). 534 patients with localised, non-MYCN amplified neuroblastoma were recruited between 1995 and 1999. Group 1 consisted of 291 patients without IDRF (Stage L1 in the International Neuroblastoma Risk Group (INRG) staging system), all treated with primary surgery. Group 2: 118 patients with IDRF (INRG Stage L2), also treated with primary surgery. Group 3: 125 patients in whom primary surgery was not attempted, 106 receiving neo-adjuvant chemotherapy. In L1 patients (Group 1) 5-year EFS was 92% and OS 98%. In L2 patients (Group 2 and 3) EFS was 79% and OS 89%. The differences in both EFS and OS were significant. EFS and OS in Group 2 (86% and 95%) were significantly better than 73% and 83% in Group 3. In INSS stage 1, 2 and 3, EFS were respectively 94%, 81% and 76%. Except between stage 2 and 3 the differences were significant. OS were respectively 99%, 93% and 83%, all significantly different. The 17% operative complication rate in L2 patients was significantly higher than 5% in L1 patients. In localised neuroblastoma, IDRF at diagnosis are associated with worse survival rates and higher rates of operative complications. The impact of IDRF should become an integrated part of therapy planning. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.
    Pediatric Blood & Cancer 02/2015; DOI:10.1002/pbc.25460 · 2.35 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Neuroblastoma (NB) is the third most common pediatric cancer. Although NB accounts for 7% of pediatric malignancies, it is responsible for more than 10% of childhood cancer-related mortality. Prognosis and treatment are determined by clinical and biological risk factors. Estimated 5-year survival rates for patients with non-high-risk and high-risk NB are more than 90% and less than 50%, respectively. Recent clinical trials have continued to reduce therapy for patients with non-high-risk NB, including the most favorable subsets who are often followed with observation approaches. In contrast, high-risk patients are treated aggressively with chemotherapy, radiation, surgery, and myeloablative and immunotherapies. Copyright © 2015 Elsevier Inc. All rights reserved.
    Pediatric Clinics of North America 02/2015; 62(1):225-256. DOI:10.1016/j.pcl.2014.09.015 · 2.20 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: This article questions the scientific justification of ingrained radiologic practices exemplified by size measurements of childhood solid tumours. This is approached by a critical review of staging systems from a selection of paediatric oncological treatment protocols. Local staging remains size-dependent for some tumour types. The consequent stage assignment can significantly influence treatment intensity. Still, the protocols tend not to give precise guidance on how to perform scans and standardise measurements. Also, they do not estimate or account for the inevitable variability in measurements. Counts and measurements of lung nodules are, within some tumour groups, used for diagnosis of metastatic disease. There is, however, no evidence that nodule size is a useful discriminator of benign and malignant lung nodules. The efficacy of imaging depends chiefly on observations being precise, accurate and valid for the desired diagnostic purpose. Because measurements without estimates of their errors are meaningless, studies of variability dependent on tumour shape and location, imaging device and observer need to be encouraged. Reproducible observations make good candidates for staging parameters if they have prognostic validity and at the same time show little covariation with (thereby adding new information to) the existing staging system. The lack of scientific rigour has made the validity of size measurement very difficult to assess. Action is needed, the most important being radiologists' active contribution in development of oncological staging systems, attention to standardisation, knowledge about errors in measurement and protection against undue influence of such errors in the staging of the individual child.
    Pediatric Radiology 01/2015; 45(1):35-41. DOI:10.1007/s00247-014-3148-0 · 1.65 Impact Factor

Full-text (2 Sources)

Download
115 Downloads
Available from
May 28, 2014