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

      Highlights

      • 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.

      Abstract

      Introduction

      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).

      Methods

      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.

      Results

      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.

      Conclusions

      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.

      Keywords

      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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