Neuroblastoma in the first year of life. The Italian contribution to a study of the Internation Society of Paediatric Oncology Europe Neuroblastoma Group

Objective. From 1999 to 2004 the SIOP Europe Neuroblastoma Group conducted a study on children with neuroblastoma diagnosed in the first year of life. The object of this report is to describe the characteristics at diagnosis of the Italian cases enrolled in this study. Methods. Patients were diagnosed in 22 Italian institutions of Paediatric Oncology participating in the Italian Cooperative Group for Neuroblastoma. Results. The total study population comprised 754 cases, and 200 (26.5%) of these were enrolled in Italy. In 67% of patients the disease was localized, in 7.5% it was disseminated and in 25.5% it was multifocal. Most tumours had favourable histological and biological characteristics. In localised disease, resection of the primary lesion was performed in the absence of surgical risk factors: 82/134 were operated radically or with minimal residue, while 52, who were considered to have an inoperable primary tumour, received neoadjuvant chemotherapy. In the disseminated disease, primary tumour resection was carried out following metastatic response, while it was avoided in multifocal disease, provided that spontaneous regression had occurred. No patient died of surgical complications. Conclusion. By following the protocol guidelines, many potentially risky interventions were postponed after chemotherapy. With this cautious behaviour, no surgical related deaths nor major surgery-related complications were observed. This achievement strengthens the importance of large-scale trials where the optimisation of care translates into greater safety for the patients.