Guido Seitz1, Cristian Urla2, Monika Sparber-Sauer3, Andreas Schuck4, Christian Vokuhl5, Bernd Blank3, Thomas Klingebiel6, Bernarda Kazanowska7, Jörg Fuchs2, Ewa Koscielniak3. 1. Department of Pediatric Surgery, University Children´s Hospital Marburg, Marburg, Germany. 2. Department of Pediatric Surgery and Urology, University Children´s Hospital, Tuebingen, Germany. 3. Klinikum Stuttgart - Olgahospital, Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin, Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart, Germany. 4. Department of Radiotherapy, University Hospital, Münster, Germany. 5. Department of Pediatric Pathology, University Hospital Schleswig-Holstein, Kiel, Germany. 6. Department of Pediatric Hematology and Oncology, University Hospital, Frankfurt/Main, Germany. 7. Department of Pediatric Bone Marrow Transplantation, Oncology and Hematology, Wroclaw Medical University, Wroclaw, Poland.
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.
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.
Authors: Martin U Schuhmann; Steven W Warmann; Cristian Urla; Jörg Fuchs; Alexander Grimm; Andreas Schmidt; Jürgen Schäfer Journal: J Cancer Res Clin Oncol Date: 2022-09-21 Impact factor: 4.322
Authors: Lianne M Haveman; Roelof van Ewijk; Elvira C van Dalen; Willemijn B Breunis; Leontien Cm Kremer; Henk van den Berg; Uta Dirksen; Johannes Hm Merks Journal: Cochrane Database Syst Rev Date: 2021-09-02
Authors: Alireza Basharkhah; Herwig Lackner; Anna Karastaneva; Marko Bergovec; Stephan Spendel; Christoph Castellani; Erich Sorantin; Martin Benesch; Bernadette Liegl-Atzwanger; Freyja-Maria Smolle-Jüttner; Christian Urban; Michael Höllwarth; Georg Singer; Holger Till Journal: Front Pediatr Date: 2021-03-15 Impact factor: 3.418