Salvage rates after progression of high-risk neuroblastoma with a soft tissue mass

Jennifer M. Murphy, Irene Isabel P. Lim, Benjamin A. Farber, Todd E. Heaton, Ellen M. Basu, Stephen S. Roberts, Shakeel Modak, Brian H. Kushner, Michael P. Laquaglia

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Purpose Treatment of progression in high-risk neuroblastoma remains challenging despite improved survival. We retrospectively evaluated outcomes in children with a first progression that included soft-tissue masses. Methods We reviewed records of 903 consecutive children with high-risk neuroblastoma diagnosed between 2004 and 2014, and identified 42 whose first progression included soft-tissue masses. Data on demographics, disease characteristics, treatment, and survival were collected. Primary outcome was 5-year overall survival (OS) from time of first progression. Secondary outcomes were local disease-free progression (LDFR) and progression-free survival (PFS) postprogression. We evaluated the prognostic significance of concomitant bone/bone marrow involvement, MYCN status, and multifocality of soft tissue relapse. Results Median age at diagnosis was 3.0 (range: 1-10.7) years. Median time to first relapse or progression was 1.2 (range: 0.1-4.5) years after complete remission or minimal stable residual disease. Twelve (29%) patients had concomitant bone or marrow involvement at progression/relapse. There were 11 (26%) patients with International Neuroblastoma Staging System stage 3 disease (all with MYCN amplification), and 31 (74%) with stage 4 disease (12 with MYCN amplification). Nine (21%) patients had multifocal soft tissue progression. R1 resection was achieved in 41 children (95%), 38 (95%) of whom also received salvage radiation therapy. Five-year OS postprogression was 35% (95% CI: 19-51%), 5-year LDFS was 52% (95% CI: 32-72%), and 5-year PFS postprogression was 20% (95% CI: 6-34%). Conclusion Among children with high-risk neuroblastoma who underwent aggressive treatment of a first soft-tissue recurrence, 5-year postprogression overall survival was 34%. Multifocality and MYCN amplification were the predominant prognostic correlates for worse survival.

Original languageEnglish (US)
Pages (from-to)285-288
Number of pages4
JournalJournal of Pediatric Surgery
Volume51
Issue number2
DOIs
StatePublished - Feb 1 2016
Externally publishedYes

Keywords

  • High-risk neuroblastoma
  • Multifocal disease
  • MYCN
  • Progression
  • Survival

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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