Japanese Treatment of High-Risk Neuroblastoma / 实用儿科临床杂志

ABSTRACT

Objective This study aimed to investigate whether more intensive induction regimen plus autologous bone marrow transplantation and 13-cis-retinoic acid can alter the extremely poor prognosis of high-risk neuroblastoma patients.Methods Five children with stage Ⅳ neuroblastoma were studied. One was under one year old, which was founded by neuroblastoma screening. The oldest one was 5 year old. The duration of illness before admission was from 7 days to 4 months. Primary site of the disease was adrenal gland in four patients and retroperitoneal in one. All of the patients had multiple metastasis. Urine VMA/Cr was more than normal in two patients. NSE elevated in all 5 patients. The MYCN amplification more than 10 copies in three patients. All of the patients received high-dose chemotherapy, radical removal of the original tumor and metastatic retroperitoneal lymph nodes, local radiation, plus supralethal preconditioning regimen followed by autologous bone marrow transplantation as well as 13-cis-retinoic acid.Results All the five patients got a complete response after the induction regimen. Follow-up 4-33 months, five patients keep complete responses and three patients disease-free survival, two patients had mild renal failure.Conclusions High-dose induction chemotherapy plus autologous bone marrow transplantation as well as 13-cis-retinoic acid may can improve the prognosis of high-risk neuroblastoma patients.