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Ewing sarcoma usually affects the bone in pediatric patients. However, extraosseous Ewing sarcoma (EES) occasionally presents in soft tissues. Specifically, visceral involvement of the gastrointestinal tract is exceptionally rare. A recent case report highlights successful pediatric visceral EES management in a 5-year-old girl. Initially, she presented with a retro-gastric mass discovered through radiological evaluation. Subsequently, histopathological analysis confirmed the diagnosis. This evaluation identified the classic t(11;22) translocation and the EWSR1-FLI1 fusion gene. Moreover, the tumor showed strong CD99 immunopositivity.
Clinicians treated the patient using a multimodal approach. First, they administered systemic chemotherapy to target the aggressive tumor. Because the lesion responded well to treatment, the surgical team then considered minimally invasive options. Consequently, they performed a laparoscopic resection of the retro-gastric tumor. This procedure was successful and avoided the morbidity associated with open laparotomy. Furthermore, the patient recovered well following the surgery. This case demonstrates that laparoscopy is a viable option for managing pediatric solid tumors after neoadjuvant therapy.
While traditional protocols favor open surgery, advanced techniques are changing the landscape. For instance, neoadjuvant chemotherapy reduces tumor volume significantly. Therefore, it creates a favorable environment for minimally invasive resection. Additionally, laparoscopy offers excellent visualization in the small pediatric abdominal cavity. Similarly, it reduces postoperative pain and hospital stays. However, clinicians must still select patients carefully based on tumor response and location. Ultimately, this rare case provides valuable evidence for the efficacy of multidisciplinary care in pediatric oncology.
The primary markers include the EWSR1-FLI1 fusion gene resulting from the t(11;22) translocation and strong CD99 immunopositivity during histopathological analysis.
Laparoscopy is significant because it provides a less invasive alternative to open surgery, reducing trauma and improving recovery for young patients while maintaining oncological safety.
Neoadjuvant chemotherapy shrinks the tumor mass, which subsequently makes it easier to achieve clear margins and facilitates the use of minimally invasive surgical techniques.
Disclaimer: This content is for informational and educational purposes only. It does not constitute medical advice or substitute for professional consultation. Refer to the latest local and national guidelines for clinical practice.
References
Khirallah MG et al. Laparoscopic resection of retro gastric extra osseous Ewing Sarcoma in a 5-year old: a rare case and review of literature. BMC Surg. 2026 May 12. doi: 10.1186/s12893-026-03793-4. PMID: 42120986.
Grier HE. The Ewing family of tumors. Pediatric Clinics of North America. 1997;44(4):991-1004.
Suryawanshi AR et al. Extraosseous Ewing’s Sarcoma of the Pancreas: A Rare Entity. Journal of Pediatric Surgery Case Reports. 2021;72:101962.

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