How is low-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

Patients with localized respectable neuroblastoma (stage 1) have excellent event-free survival (EFS) rates with surgical excision of tumor only. Adjuvant chemotherapy is generally not needed for this group of patients. Even the presence of residual microscopic disease does not significantly affect the EFS. If patients develop recurrent disease, chemotherapy can be used, and the overall survival rate remains higher than 95%.

Similar therapy is offered to patients with stage 2A/2B disease who are presently assigned to a low-risk category if they have MYCN -non amplified tumors, regardless of age histology or ploidy. Patients with stage 2A/2B disease with amplified MYCN are considered high risk regardless of age and histology.

A study by the Pediatric Oncology Group of experience with conservative treatment of low-risk patients confirmed the excellent outcomes for these patients with surgery alone. However, overall survival seemed lower among patients with stage 2b, MYC-N nonamplified, unfavorable histology or diploid tumors; thus, in the future, this specific group of patients may require reconsideration of their risk categorization. [20]

Most patients with 4S disease (ie, non-MYCN –amplified tumors, favorable histology, hyperdiploid tumors in infants younger than 1 y) are also considered to be in the low-risk group and most experience spontaneous regression. Thus, observation or surgery alone is often all that is needed to manage these tumors. Chemotherapy may be used to control life-threatening situations such as respiratory distress or mechanical obstruction.


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