Zentralbl Chir 2022; 147(S 01): S73-S74
DOI: 10.1055/s-0042-1754256
Abstracts
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Primary mediastinal Ewing's Sarcoma: Post hoc analysis from two international multicenter prospective randomized trials

T Stork
1   Ruhrlandklinik – Universitätsmedizin Essen, Abteilung für Thoraxchirurgie und thorakale Endoskopie, Essen, Deutschland
,
A Ranft
2   Universitätsklinikum Essen, Institut für Pathologie, Essen, Deutschland
,
C Aigner
1   Ruhrlandklinik – Universitätsmedizin Essen, Abteilung für Thoraxchirurgie und thorakale Endoskopie, Essen, Deutschland
,
U Dirksen
2   Universitätsklinikum Essen, Institut für Pathologie, Essen, Deutschland
,
S Collaud
1   Ruhrlandklinik – Universitätsmedizin Essen, Abteilung für Thoraxchirurgie und thorakale Endoskopie, Essen, Deutschland
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    Hintergrund Mediastinal soft tissue sarcomas (STS) represent about 1% of all soft tissue sarcomas. Reported survival is poor with 5-year overall survival of about 30%. Ewing´s sarcoma (ES) of the mediastinum is extremely rare, with only few cases reported in the literature. Here we aimed to gain better understanding of primary mediastinal ES in describing patients treated within two international multicenter prospective randomized ES trials.

    Material und Methode Data from patients with primary mediastinal ES were retrieved from database of the EURO-E.W.I.N.G.99 (ClinicalTrials.gov identifier: NCT00020566) and EWING-2008 (ClinicalTrials.gov identifier: NCT00987636) trials. Patient and treatment characteristics were analyzed.

    Ergebnis Out of 2969 patients with ES, nine (0.3%) had primary mediastinal ES. Median age at diagnosis was 30.5 years (4 to 49). Eight (89%) patients had biopsy prior to multimodal treatment, while one (11%) had upfront surgery. At the time of diagnosis, three patients had synchronous metastases to the lung (n=1, 11%), bone (n=1, 11%) or lung and bone (n=1, 11%). All patients underwent multiagent chemotherapy consisting of vincristine, ifosfamide, actinomycin D, etoposide (VIDE) and vincristine, actinomycin D, ifosfamide (VAI) in most patients (n=5, 55%).

    Local therapy for non-metastatic primary mediastinal ES was surgery alone (n=2, 22%), combined surgery and radiotherapy (n=2, 22%), radiation alone (n=1, 11%) or none (n=1, 11%). Surgery consisted in extended resections in most patients (n=3, 33%) including resection of lung parenchyma, pericardium, oesophageal muscle layer, atrium and diaphragm. Median follow-up was 170 months (18-293). Overall 5-year survival for the whole cohort was 64%. Beside one patient who was lost of follow-up, all patients who had surgery, were alive at the end of follow-up.

    Schlussfolgerung Primary mediastinal ES is extremely rare. Surgery can provide excellent long-term outcome when feasible.


     

    Publication History

    Article published online:
    13 September 2022

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