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Malignant peripheral nerve sheath tumors in children, adolescents, and young adults: Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
Author(s) -
Meister Michael T.,
Scheer Monika,
Hallmen Erika,
Stegmaier Sabine,
Vokuhl Christian,
Kalle Thekla,
Fuchs Jörg,
Münter Marc,
Niggli Felix,
Ladenstein Ruth,
Kazanowska Bernarda,
Ljungman Gustaf,
Bielack Stefan,
Koscielniak Ewa,
Klingebiel Thomas
Publication year - 2020
Publication title -
journal of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.201
H-Index - 111
eISSN - 1096-9098
pISSN - 0022-4790
DOI - 10.1002/jso.26153
Subject(s) - medicine , neurofibromatosis , rhabdomyosarcoma , malignant peripheral nerve sheath tumor , soft tissue sarcoma , prospective cohort study , univariate analysis , sarcoma , clinical trial , oncology , cohort , soft tissue , surgery , pathology , multivariate analysis
Background and Objectives Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas that present as large, invasive tumors. Our aim was to assess outcomes, identify prognostic factors, and analyze treatment strategies in a prospectively collected pediatric cohort. Methods Patients less than 21 years with MPNST treated in the consecutive prospective European Cooperative Weichteilsarkom Studiengruppe (CWS)‐trials (1981‐2009) and the CWS‐SoTiSaR registry (2009‐2015) were analyzed. Results A total of 159 patients were analyzed. Neurofibromatosis type I (NF1) was reported in thirty‐eight patients (24%). Most were adolescents (67%) with large (>10 cm, 65%) tumors located at extremities (42%). Nodal involvement was documented in 15 (9%) and distant metastases in 15 (9%) upon diagnosis. Overall, event‐free survival (EFS) was 40.5% at 5 and 36.3% at 10 years, and overall survival (OS) was 54.6% at 5 and 47.1% at 10 years. Age, NF1 status, tumor site, tumor size, Intergroup Rhabdomyosarcoma Study (IRS) group, metastatic disease, and achieving first complete remission (CR1) were identified as prognostic factors for EFS and/or OS in the univariate analysis. Conclusions Prognostic factors were identified and research questions for future clinical trials were addressed.