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. (8th August 2019)

• Record Type: Journal Article
• Authors: Seitz, Guido; Urla, Cristian; Sparber‐Sauer, Monika; Schuck, Andreas; Vokuhl, Christian; Blank, Bernd; Klingebiel, Thomas; Kazanowska, Bernarda; Fuchs, Jörg; Koscielniak, Ewa
• Other Names: Shamberger Robert C. guestEditor.
• Is Part Of: Pediatric blood & cancer. Volume 66(2019)Supplement 3
• Page Start: n/a
• Page End: n/a
• Publication Date: 2019-08-08
• Subjects: Ewing sarcoma -- local control -- soft‐tissue sarcoma -- thorax; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.27884
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: 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.