Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors

Luca Bergamaschi, Gianni Bisogno, Carla Manzitti, Paolo D'Angelo, Giuseppe Maria Milano, Angela Scagnellato, Mirko Cappelletti, Stefano Chiaravalli, Patrizia Dall'Igna, Rita Alaggio, Antonio Ruggiero, Martina Di Martino, Maria Carmen Affinita, Marta Pierobon, Alberto Garaventa, Michela Casanova, Andrea Ferrari

Research output: Contribution to journalArticlepeer-review


Background: Malignant peripheral nerve sheath tumor (MPNST) is one of the most common nonrhabdomyosarcoma soft tissue sarcomas encountered in pediatric age, and it is generally characterized by poor outcome, particularly for relapsing patients. Materials and methods: This study considered 73 patients <21 years of age with relapsing MPNST observed among 120 patients enrolled in Italian pediatric protocols from 1979 to 2004. With the aim of possibly establishing a risk-adapted stratification, patients’ outcome was examined using univariate and multivariate analysis based on clinical features at onset, first-line treatments, clinical findings at the time of first relapse, and second-line treatments. Results: The time to relapse ranged from 1 to 204 months after first diagnosis (median 7 months). The first relapse event was mainly local. At the time of our analysis, nine patients were alive in remission. The median overall survival after first relapse was 11 months, and the survival rates were 39.2% at 1 year and 15.8% at 5 years. The factors revealing the greatest impact on prognosis were as follows: initial tumor invasiveness, time of relapse, and achievement of a secondary complete remission (which was related to the feasibility of radical surgery). Conclusions: Our study confirmed the unsatisfactory prognosis for pediatric patients with relapsing MPNST and pointed to a risk-adapted stratification model for the purposes of deciding second-line treatments. For the time being, an aggressive surgical approach seems to be the only effective salvage treatment and should be recommended. New therapeutic approaches are under evaluation with a view to improving current outcomes.

Original languageEnglish
Article numbere26816
JournalPediatric Blood and Cancer
Issue number2
Publication statusPublished - Sep 19 2017


  • children and adolescents
  • malignant peripheral nerve sheath tumors
  • nonrhabdomyosarcoma soft tissue sarcomas
  • pediatric soft tissue sarcomas
  • prognostic factors
  • relapse
  • salvage rate

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology


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