How is intermediate-risk pediatric neuroblastoma treated?

Updated: Oct 09, 2017
  • Author: Norman J Lacayo, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Answer

Surgery and multiagent chemotherapy comprise the backbone of therapy for intermediate risk group patients. Current efforts are ongoing to help understand which of this diverse group of patients can have therapy reduced without threatening the excellent EFS for these patients.

Intermediate-risk patients include children younger than 18 months with stage 3 and 4 disease and favorable biology (non-MYCN –amplified tumors, regardless of histology and DNA index). These patients are offered therapy with 4 of the most active drugs against neuroblastoma (ie, cyclophosphamide, doxorubicin, carboplatin, etoposide) for either 4 cycles, 6 cycles, or 8 cycles, depending on histology and DNA index and response to treatment. In these patients, surgery can be performed either at time of diagnosis or following multiagent chemotherapy. If residual disease is present after chemotherapy and surgery, radiation therapy could be considered. However, the use of radiation is controversial, although a POG study suggested that it improves outcome when administered to areas of residual disease postchemotherapy.

Baker et al conducted a prospective, phase 3, nonrandomized trial of 479 patients (270 patients with stage 3 disease, 178 patients with stage 4 disease, and 31 patients with stage 4S disease) to determine whether a 3-year estimated overall survival of more than 90% could be maintained with reduced duration of chemotherapy and reduced drug doses. [21] The resulting 3-year estimate of overall for the entire group was 96%±1%. The study concluded that among patients with intermediate-risk neuroblastoma, substantially reduced duration of chemotherapy and reduced doses of chemotherapeutic agents still resulted in excellent outcomes.


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