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)To read this article in full you will need to make a payment
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Article info
Publication history
Accepted:
January 9,
2023
Received in revised form:
December 21,
2022
Received:
November 29,
2022
Publication stage
In Press Accepted ManuscriptIdentification
Copyright
© 2023 Elsevier Inc. All rights reserved.
