Total-body sequential segmental irradiation and combination chemotherapy for children with disseminated neuroblastoma

Fourteen children with disseminated neuroblastoma were treated with segmental total-body irradiation and a combination of cyclophosphamide, vincristine and Adriamycin. Six others had localized tumors and were treated with either surgery alone or limited irradiation and chemotherapy. The main objective of the study was to find out if this aggressive therapy for disseminated neuroblastoma would be tolerated and would promote longer disease-free remissions and possibly, cures. Complete responses were obtained in all patients with localized tumors. Of the 14 patients with disseminated disease, three had complete responses, five had partial responses, two had objective responses, and four were classified as nonresponders. Survival rates for this group are poor. Only two patients are still alive, and the single patient who survives free of disease has had a complex clinical course that is only indirectly a result of treatment. The median survival for patients with disseminated tumor was 9 months, not appreciably different from earlier result. Although toxicity was clinically manageable, it interfered with the planned schedule of therapy. Continuous weight loss combined with severe bone marrow suppression due to irradiation prevented about 50% of the scheduled drug dose from being administered. This resulted in poor control of tumor outside of radiation ports and eventually led to progressive disease within previously irradiated sites. We conclude that irradiation used in this manner will not improve disease-free survival of children with disseminated neuroblastoma. It is essential that disseminated disease be controlled before extensive irradiation is attempted.