Prospective evaluation of the International Neuroblastoma Staging System (INSS) and the International Neuroblastoma Response Criteria (INRC) in a multicentre setting

The aim of this study was to classify prospectively a series of neuroblasto ma tumours according to the International Neuroblastoma Staging System (INS S) and the International Neuroblastoma Response Criteria (INRC) and to eval uate the difficulties and pitfalls involved in a multicentre setting. Each hospital provided their data for central review. The surgical procedures an d their complications were reported. Kaplan-Meier estimates of survival and event-free survival were calculated according to stage and response to the rapy. From June 1992 to December 1996, 194 patients were included in the st udy, with a mean age of 2 years. Initial studies were performed according t o INSS recommendations without major problems. INSS stage was correctly app lied to all patients except for 9 (95%). Postoperative complications were o bserved in 15 patients (8.3%). Response to therapy (INRC) was studied in 63 stage 4 patients, 11 of whom were not classified correctly (17%). Differen ces in survival according to stage (INSS) and group of response to therapy (INRC) were statistically significant (P < 0.001). In conclusion the INSS w as easy to use and separated different prognostic groups. Surgical complica tions and mortality did not increase in this series because of using the IN SS. The feasibility of INRC was evaluated in a small series of stage 4 pati ents and the designation of response was problematic in a relatively high p roportion of cases. The prognostic value of the different responses was hig hly significant, but less informative than had been hoped for. (C) 1999 Els evier Science Ltd. All rights reserved.