Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1.

Abstract:

BACKGROUND:Patients affected by neurofibromatosis type 1 (NF1) are at higher risk of developing soft-tissue sarcomas (STS) than the general population. The clinical findings and outcome in 43 children and adolescents with NF1 treated for STS in the Italian protocols between 1988 and 2004 are reported. METHODS:The study included 37 patients with neurogenic sarcomas (36 malignant peripheral nerve sheath tumors [MPNST], 1 triton tumor) and 6 cases of rhabdomyosarcoma (RMS). The prevalence of NF1 observed during the study period was 43% in the MPNST population and 1% in the RMS group. RESULTS:Most patients with neurogenic sarcomas had large, invasive tumors. Five-year event-free and overall survival rates were 19% and 28%, respectively. Two of 16 patients with evaluable disease responded to chemotherapy. All 6 RMS patients were

journal_name

Cancer

journal_title

Cancer

authors

Ferrari A,Bisogno G,Macaluso A,Casanova M,D'Angelo P,Pierani P,Zanetti I,Alaggio R,Cecchetto G,Carli M

doi

10.1002/cncr.22533

subject

Has Abstract

pub_date

2007-04-01 00:00:00

pages

1406-12

issue

7

eissn

0008-543X

issn

1097-0142

journal_volume

109

pub_type

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