LOCALIZED RESECTABLE NEUROBLASTOMA - RESULTS OF THE 2ND STUDY OF THE ITALIAN COOPERATIVE GROUP FOR NEUROBLASTOMA

Purpose: To optimize treatment for children with localized resectable neuroblastoma in 21 Italian institutions using a common protocol based on previous experience. Patients and Methods: Between January 1985 an d December 1992, 152 children aged 0 to 15 years with nondisseminated neuroblastoma were entered onto this study following complete resectio n of tumor without tumar rupture (TR) (stage 1), or resection with min imal tumor residue, and/or tumor infiltration of regional lymph nodes (LN+), and/or TR (stage 2). Of 144 assessable children, 69 were classi fied as having stage 1 disease and 75 as stage 2. Of stage 2 children, 49 had low-risk (LR) characteristics (age, 0 to 11 months or 1 to 15 years but negative lymph nodes and no TR). Stage 1 and stage 2 LR chil dren did not receive adjuvant therapy. The remaining 26 stage 2 childr en had high-risk (HR) characteristics (age, 1 to 15 years with LN+ and /or TR) and received adjuvant chemotherapy for 6 months. Results: Of 1 44 children, three died of therapy-related complications and 19 relaps ed, of whom six died of disease. The estimated 5-year overall survival (OS) rate was 93% and the event-free survival (EFS) rate was 83%. Of 69 stage 1 children, one died postoperatively and five relapsed (one l ocal and four disseminated, two of whom died), for 94% OS and 90% EFS rates. Of 49 stage 2 LR children, six relapsed (four local and two dis seminated); relapses occurred in five of 20 infants with LN+, in one o f four infants with TR, and in none of the remaining 25 children. One child died of disease and one of toxicity, for 96% OS and 85% EFS rate s. Of 26 stage 2 HR children, eight relapsed (three of 20 with LN+, th ree of four with TR, and two of two with LN+ and TR), of whom three di ed of disease and one of toxicity, for 87% OS and 61% EFS rates. Concl usion: Our data confirm the overall good prognosis of children with lo calized resectable neuroblastoma. LN+ and TR predisposed to relapse at all ages, but infants tended to have a less aggressive course after r elapse. Stage 1 and 2 LR children had 94% and 96% OS rates, respective ly, which justifies a policy of no adjuvant chemotherapy. Eight of 26 children with stage 2 HR relapsed despite 6 months of chemotherapy; fo r these children, more intensive chemotherapy may be required. J Clin Oncol 13:884-893. (C) 1995 by American Society of Clinical Oncology.