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.
