Successful treatment of infants with localized neuroblastoma based on their MYCN status

Int J Clin Oncol. 2013 Jun;18(3):389-95. doi: 10.1007/s10147-012-0391-y. Epub 2012 Mar 2.

Abstract

Background: The aim of this study was to evaluate the effectiveness of post-surgical chemotherapy for infants with localized neuroblastoma without MYCN amplification (MNA), and determine whether risk classification using MNA is reasonable.

Methods: Four hundred and fourteen eligible patients were registered between 1998 and 2004. Resectable patients in stage 1 and 2A/2B were treated by surgical resection only. Unresectable patients in stage 3 without MNA received either 6 cycles of regimen A or 3 cycles of regimen A plus 3 cycles of regimen C2; regimen A consisted of low doses of cyclophosphamide and vincristine and regimen C consisted of cyclophosphamide, vincristine and pirarubicin before surgical resection. The resectable and unresectable patients were randomly selected to receive post-surgical chemotherapy. The patients with MNA received intensive chemotherapy regimen D2, consisting of cyclophosphamide, vincristine, pirarubicin and cisplatin, and some of them received high-dose chemotherapy with stem cell transplantation.

Results: The 5-year event-free survival (5-EFS) rates of stage 1 and 2A/2B patients without MNA were 97.2 and 89.0% respectively (p = 0.02). A total of 31 patients in stage 3 without MNA received post-surgical chemotherapy, and 30 patients did not. The 5-EFS rates of these two groups (96.0 and 96.2%, respectively) were not significantly different (p = 0.869). The 5-EFS rate for localized patients with MNA (n = 6) was 50.0%, and that of patients without MNA was 95.0% (p < 0.001).

Conclusion: Post-surgical chemotherapy was therefore unnecessary for localized patients without MNA. This treatment strategy using MNA is considered to be appropriate in infants.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cisplatin / administration & dosage
  • Cyclophosphamide / administration & dosage
  • Disease-Free Survival
  • Doxorubicin / administration & dosage
  • Doxorubicin / analogs & derivatives
  • Gene Expression Regulation, Neoplastic
  • Humans
  • Infant
  • N-Myc Proto-Oncogene Protein
  • Neoplasm Staging
  • Neuroblastoma / drug therapy*
  • Neuroblastoma / genetics*
  • Neuroblastoma / pathology
  • Neuroblastoma / surgery
  • Nuclear Proteins / biosynthesis*
  • Nuclear Proteins / genetics
  • Oncogene Proteins / biosynthesis*
  • Oncogene Proteins / genetics
  • Prognosis
  • Vincristine / administration & dosage

Substances

  • MYCN protein, human
  • N-Myc Proto-Oncogene Protein
  • Nuclear Proteins
  • Oncogene Proteins
  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • pirarubicin
  • Cisplatin