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Treatment and outcome of patients with thoracic tumors of the Ewing sarcoma family: A report from the Cooperative Weichteilsarkom Studiengruppe CWS‐81, ‐86, ‐91, ‐96, and ‐2002P trials
Author(s) -
Seitz Guido,
Urla Cristian,
SparberSauer Monika,
Schuck Andreas,
Vokuhl Christian,
Blank Bernd,
Klingebiel Thomas,
Kazanowska Bernarda,
Fuchs Jörg,
Koscielniak Ewa
Publication year - 2019
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.27537
Subject(s) - medicine , sarcoma , soft tissue sarcoma , overall survival , clinical trial , retrospective cohort study , surgery , pathology
Background Ewing tumors are the most frequent malignant tumors of the chest wall in children and young adults. Surgical management of these tumors can be challenging. Optimal local control remains controversial. The aim of this study was to analyze treatment, outcome, and surgical procedures in patients with thoracic tumors of the Ewing sarcoma family (TES) treated within four Cooperative Soft‐Tissue Sarcoma (CWS) trials and one registry. Patients and methods Sixty‐two patients from 0 to 21 years treated between 1981 and 2014 were selected for this analysis. A retrospective chart analysis was carried out. Institutional review board approval was obtained for all trials. Results The median age of the patients was 7 years. The 5‐year overall (OS) and event‐free survival (EFS) rates were 58.7% (52.7–64.7) and 52.8% (46.8–58.8). Patients with intrathoracic tumor localization ( n = 24) had a worse outcome (EFS: 37.5%; 27.5–37.5) compared with those with chest wall tumors ( n = 38; EFS: 62.3%; 54.3–70.3, P = 0.008). Patients ≤10 years ( n = 38) had a better survival compared with those > 10 years (EFS: 65.7%; 57.7–73.7 vs 31.3%; 21.3–41.3, P = 0.01). Tumor size ≤5 cm ( n = 15) was associated with significantly better survival compared with a size > 5 cm ( n = 47, EFS: 93.3%; 87.3–99.3 vs 40%; 33‐47, P = 0.002). Primary resections were carried out in 36 patients, of which 75% were incomplete resulting in inferior EFS ( P = 0.006). Complete secondary resections were performed in 22 of 40. Conclusions Positive predictive factors for outcome are age ≤10 years, size ≤5 cm, and localization at the chest wall. Diverse IRS groups require individual treatment.