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. 2023 Sep;58(9):1727-1735.
doi: 10.1016/j.jpedsurg.2023.01.006. Epub 2023 Jan 14.

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

Marshall W Wallace  1 , Jan A Niec  2 , Muhammad Owais Abdul Ghani  3 , Katlyn G McKay  4 , Kamran Idrees  5 , Jiancong Liang  6 , Scott C Borinstein  7 , Harold N Lovvorn 3rd  8
Affiliations

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

Marshall W Wallace et al. J Pediatr Surg. 2023 Sep.

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 and 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: Ewing sarcoma; Ewing-like; Surgical management; Survival outcomes; Visceral ewing sarcoma.

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