Treatment results of advanced neuroblastoma with the first Japanese study group protocol

Purpose: To elucidate the efficacy of intensive induction and consolidation chemotherapy regimens (Study Group of Japan for Advanced Neuroblastoma [JA NB] 85) for patients with advanced neuroblastoma aged 1 year or older. Patient and Methods: One hundred fifty-seven patients with newly diagnosed advanced neuroblastoma were entered into this study between January 1985 an d December 1990. Eligible patients were 12 months old or older with stage I II or IV disease. The patients first received six cyclic courses of intensi ve induction chemotherapy (designated regimen Al) consisting of cyclophosph amide (1,200 mg/m(2)), vincristine (1.5 mg/m(2)), tetrahydro-pyranyl Adriam ycin (pirarubicin; 40 mg/m2), and cisplatin (90 mg/m2). The patients were f urther treated with three different consolidation protocols: 3-[(4-amino-2- methyl-5-pyrimidinyl)methyl]-1-(2-chloroethyl)-1-nitrosourea, dacarbazine, and bone marrow transplantation. Results: Overall survival rates for patients with stage III disease without reference to the consolidation protocols were 80.8%. 76.9% and 66.3% at 2, 5, and 10 years, respectively. The overall survival rates for patients wit h stage IV disease were 58.8%, 34.4%, and 28.9% at 7, 5, and 10 years, resp ectively. There were no statistically significant differences between the t hree consolidation treatment groups. Patients who did not achieve complete remission (CR) with induction chemotherapy and surgery all died, suggesting that CR is essential for the curl of advanced neuroblastoma. The overall 5 -year survival rate of the 24 patients with N-myc amplified stage III and I V disease was 33.3%, and the longest survival time of a relapse-free patien t was 103 months. Conclusion: The intensive induction chemotherapy regimen used in this study may be of significant value in increasing the CR rate and survival for pat ients with N-myc amplified and nonamplified advanced neuroblastoma.