Distribution and Surgical Management of Visceral Ewing Sarcoma Among Children and Adolescents


      • Unlike osseous Ewing Sarcoma, visceral Ewing Sarcoma is poorly described, with a paucity of data available regarding its presentation, outcomes, and management.
      • Visceral Ewing Sarcoma requires nuanced and unique surgical primary control strategies but maintains similar survival when treated with similar overall principles as other forms of EWS.



      Ewing sarcoma (EWS) is a highly malignant tumor of bone and soft tissue that occasionally arises from viscera. Visceral EWS (V-EWS) is challenging to manage given its varied organ distribution and often late-stage presentation. We aimed to characterize our institutional experience with V-EWS, focusing on its surgical management, and to compare V-EWS outcomes against those with osseous (O-EWS) and soft tissue EWS (ST-EWS).


      Retrospective review of all EWS patients ≤21 years presenting to a single institution between 2000-2022. Patient- and disease-specific characteristics were compared. Overall and relapse-free survival were estimated using Kaplan Meier methods and log-rank test.


      156 EWS patients were identified: 117 O-EWS, 20 ST-EWS, and 19 V-EWS. V-EWS arose in the kidney (n=5), lung (n=5), intestine (n=2), esophagus (n=1), liver (n=1), pancreas (n=1), adrenal gland (n=1), vagina (n=1), brain (n=1), and spinal cord (n=1). No significant demographic differences were detected between EWS groups. V-EWS was more frequently metastatic at presentation (63.2%; p=0.005), yet no significant overall or relapse-free survival differences emerged between EWS groups, with similar follow-up intervals. While V-EWS required multiple unique operative strategies to gain primary control, no significant difference in treatment strategies appeared between groups. Surgery-only primary control was associated with improved overall and relapse-free survival in all groups.


      V-EWS presents unique management challenges in children and adolescents given its variable sites of origin. This large cohort is the first to describe the surgical management and outcomes of V-EWS, demonstrating more frequent metastatic presentation, while achieving similar survival across groups.

      Level of Evidence

      Level 2 – Cohort Study.


      Abbreviations used:

      EWS (Ewing Sarcoma), O-EWS (Osseous Ewing Sarcoma), ST-EWS (Soft-tissue Ewing Sarcoma), V-EWS (Visceral Ewing Sarcoma), EMR (Electronic Medical Record)
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