TY - JOUR
T1 - Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors
AU - Bergamaschi, Luca
AU - Bisogno, Gianni
AU - Manzitti, Carla
AU - D'Angelo, Paolo
AU - Milano, Giuseppe Maria
AU - Scagnellato, Angela
AU - Cappelletti, Mirko
AU - Chiaravalli, Stefano
AU - Dall'Igna, Patrizia
AU - Alaggio, Rita
AU - Ruggiero, Antonio
AU - Di Martino, Martina
AU - Affinita, Maria Carmen
AU - Pierobon, Marta
AU - Garaventa, Alberto
AU - Casanova, Michela
AU - Ferrari, Andrea
PY - 2017
Y1 - 2017
N2 - 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.
AB - 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.
KW - Children and adolescents
KW - Hematology
KW - Malignant peripheral nerve sheath tumors
KW - Nonrhabdomyosarcoma soft tissue sarcomas
KW - Oncology
KW - Pediatric soft tissue sarcomas
KW - Pediatrics
KW - Perinatology and Child Health
KW - Prognostic factors
KW - Relapse
KW - Salvage rate
KW - Children and adolescents
KW - Hematology
KW - Malignant peripheral nerve sheath tumors
KW - Nonrhabdomyosarcoma soft tissue sarcomas
KW - Oncology
KW - Pediatric soft tissue sarcomas
KW - Pediatrics
KW - Perinatology and Child Health
KW - Prognostic factors
KW - Relapse
KW - Salvage rate
UR - https://publicatt.unicatt.it/handle/10807/105727
UR - https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=85030236502&origin=inward
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85030236502&origin=inward
U2 - 10.1002/pbc.26816
DO - 10.1002/pbc.26816
M3 - Article
SN - 1545-5009
VL - 65
SP - N/A-N/A
JO - PEDIATRIC BLOOD & CANCER
JF - PEDIATRIC BLOOD & CANCER
IS - 2
ER -