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. 2025 Jan 2;17(1):118.
doi: 10.3390/cancers17010118.

Primary Mediastinal Ewing's Sarcoma: Post Hoc Analysis from Two International Multicenter Prospective Randomized Trials

Theresa Stork  1   2   3 , Andreas Ranft  3   4 , Clemens Aigner  1   3   5 , Heribert Jurgens  6 , Ruth L Ladenstein  7 , Beate Timmermann  3   8 , Henk Van den Berg  9 , Uta Dirksen  3   4 , Stéphane Collaud  1   2   3
Affiliations

Primary Mediastinal Ewing's Sarcoma: Post Hoc Analysis from Two International Multicenter Prospective Randomized Trials

Theresa Stork et al. Cancers (Basel). .

Abstract

Objective: Ewing sarcoma (EWS) of the mediastinum is extremely rare, with only a few cases reported in the literature. We aimed to gain a better understanding of primary mediastinal EWS, describing patients treated within two international, multicenter, prospective, randomized EWS trials.

Methods: Data from patients with primary mediastinal EWS were retrieved from the 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.

Results: Out of 2969 patients with EWS, 9 (0.3%) had primary mediastinal EWS. The median age at diagnosis was 30.5 years (4 to 49). At the time of diagnosis, n = 3 (33%) patients had synchronous metastases. All patients underwent multiagent chemotherapy. Local therapy for non-metastatic patients was surgery (n = 2, 22%), surgery and radiotherapy (n = 2, 22%) or radiotherapy alone (n = 2, 22%). Surgery consisted of extended resections in most patients (n = 3, 33%). Five-year survival for the whole cohort was 64%. Apart from one patient who was lost to follow-up, all patients (n = 4) who had undergone surgery were alive at the end of follow-up.

Conclusions: Primary mediastinal ES is extremely rare, with a prevalence of 0.3% among EWS. Five-year survival was favorable compared to primary mediastinal sarcoma of all histologies and in line with EWS of different origin.

Keywords: Ewing sarcoma; mediastinal; multimodal therapy; sarcoma; soft tissue sarcoma.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Depicts the variability in appearance of Ewing sarcoma on CT imaging. (A) Shows an unresectable tumor located in the middle mediastinum and diffusely infiltrating the heart. (B) Shows a small, localized tumor of the anterior mediastinum.
Figure 2
Figure 2
Overall survival for patients with primary mediastinal Ewing sarcoma.
See this image and copyright information in PMC

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