# Renal ewing sarcoma in a young female: a case report and review of targeted therapy

**Authors:** Pengfei Wang, Mingfa Wang, Jiangtao Zhan, Xinming Hu, Xusong Meng

PMC · DOI: 10.3389/fsurg.2025.1512474 · Frontiers in Surgery · 2025-03-04

## TL;DR

This paper reports a rare case of renal Ewing sarcoma in a young woman and discusses treatment approaches and targeted therapy developments.

## Contribution

The paper presents a rare case of renal Ewing sarcoma and explores the integration of next-generation sequencing with targeted therapy for improved treatment.

## Key findings

- Renal Ewing sarcoma is extremely rare, with less than 1% of all renal malignancies being this type.
- The patient's tumor had P53 and STGA2 mutations, and the disease progressed to the pancreas despite treatment.
- Combining next-generation sequencing with targeted therapy is suggested as a potential approach for treating RES.

## Abstract

Ewing sarcoma (ES) is an aggressive neoplasm predominantly affecting pediatric and adolescent populations. Renal involvement in ES is exceedingly rare, representing less than 1% of all renal malignancies. Herein, we present the case of a 22-year-old female diagnosed with renal Ewing sarcoma (RES) accompanied by renal vein thrombosis. The patient reported a one-month history of persistent left lumbar pain, prompting hospitalization. Magnetic resonance imaging identified an extensive left suprarenal mass measuring 13.5 × 10.5 × 4.5 cm, with concurrent renal vein thrombosis. The comprehensive evaluation of histopathology, immunohistochemistry and molecular genetics confirmed RES. The treatment included radical left nephrectomy, followed by adjuvant chemotherapy (i.e., vincristine, epirubicin and cyclophosphamide) after surgery. Genetic analysis of the tumor revealed mutations in P53 and STGA2. Follow-up contrast-enhanced computed tomography scans of the patient demonstrated metastatic progression to the pancreas. The patient passed away after a 7-month follow-up period. This article reviews our treatment experience and recent developments in targeted therapies. Aiming to provide new approaches for the treatment of RES, this combines next-generation sequencing technology with targeted therapy to promote the optimization of targeted treatments.

## Linked entities

- **Genes:** TP53 (tumor protein p53) [NCBI Gene 7157]
- **Chemicals:** vincristine (PubChem CID 5978), epirubicin (PubChem CID 41867), cyclophosphamide (PubChem CID 2907)
- **Diseases:** Ewing sarcoma (MONDO:0012817)

## Full-text entities

- **Genes:** TP53 (tumor protein p53) [NCBI Gene 7157] {aka BCC7, BMFS5, LFS1, P53, TRP53}
- **Diseases:** ES (MESH:D012512), Renal involvement (MESH:C565423), neoplasm (MESH:D009369), renal vein thrombosis (MESH:D012170), lumbar pain (MESH:D010146)
- **Chemicals:** epirubicin (MESH:D015251), vincristine (MESH:D014750), cyclophosphamide (MESH:D003520)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11913866/full.md

## References

41 references — full list in the complete paper: https://tomesphere.com/paper/PMC11913866/full.md

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Source: https://tomesphere.com/paper/PMC11913866