Surgery in pediatric solid tumors with special reference to advanced neuroblastoma.

UNLABELLED: The role of surgery in Wilms' tumor and hepatoblastoma is well established, but that in advanced neuroblastoma is controversial. We analyzed factors contributing to the long-term survival of patients with MYCN-amplified neuroblastoma.

METHOD: Patients with stage 3, 4, and 4S neuroblastoma with more than 10 copies of MYCN received induction regimen A1 from January 1985 to February 1991, and with regimen A3 which contains twice the dose of cyclophosphamide from March 1991 to September 1993. Most of these patients underwent radical removal of the original tumor and metastatic lymph nodes plus supralethal preconditioning regimens followed by autologous bone marrow transplantation (ABMT) or peripheral blood stem cell transplantation (PBSCT). In assessing the radicality of surgery, three categories of "total", "subtotal", and 'partial" were used.

RESULTS

During the study period, 66 patients with more than 10 copies of MYCN were treated, and 19 (29%) of the 66 patients survived disease-free for at least 69 months. All but one who survived, for more than 69 months disease-free underwent ABMT or PBSCT The radicality of surgery was total in 18 (95%), and subtotal in 1 (5%) of the 19 long-term survivors, while it was total in 38 (84%), and subtotal in 7 (16%) of the 45 patients who died (p > 0.05).

CONCLUSIONS

Nineteen (29%) of the 66 patients with MYCN amplification were long-term disease-free survivors for more than 69 months. The prerequisites for survival in such patients appear to be intensive chemotherapy, total resection of the tumor plus metastases, and the use of ABMT/PBSCT, without any major delay in the time sequence.