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Published in: BMC Urology 1/2022

Open Access 01-12-2022 | Nephrectomy | Case report

Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour of the kidney: a case report and literature review

Authors: Jing Li, Fang Nie, Yan Li

Published in: BMC Urology | Issue 1/2022

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Abstract

Background

Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumours(EWS/pPNETs) of the kidney are rare. Signs and symptoms are atypical in EWS patients. Presenting symptoms include haematuria, abdominal pain, or a palpable mass. A comprehensive review of the literature shows that it is difficult to make an accurate diagnosis based on physical examination alone. The imaging findings of EWS/pPNETs are nonspecific. We used contrast-enhanced ultrasound (CEUS) to diagnose an EWS/pPNET in our patient, which had never been reported previously to our knowledge.

Case presentation

This article reports the case of a 20-year-old female with an abdominal mass and gross haematuria for 1 month. The ultrasound revealed a hypoechoic mass with a clear margin at the lower pole in the left kidney. CEUS demonstrated signs of annular enhancement and heterogeneous enhancement of the tumour, and simultaneous wash-in was predominant. Computed tomography images showed an elliptical low-density tumour. The patient underwent a left kidney resection, and the pathological diagnosis was an EWS/pPNET. Twenty-one days after the kidney operation, the patient underwent 8 cycles of a CAV (vinorelbine, ifosfamide, epirubicin) + IE (isocyclophosphamide, etoposide) chemotherapy regimen. Subsequently, radiotherapy (dose: 45 Gy, radiation field:the tumour bed following surgical resection) was administered for nearly 30 days. The patient had no signs of local recurrence or metastasis within a follow-up of 4 years.

Conclusions

As a radiation-free, inexpensive, convenient, and repeatable examination method, ultrasound was the primary choice for kidney examination. Early CEUS was helpful to make an accurate diagnosis. Surgery and adjuvant radiation or chemotherapy administered in a timely manner can prevent further deterioration.
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Metadata
Title
Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour of the kidney: a case report and literature review
Authors
Jing Li
Fang Nie
Yan Li
Publication date
01-12-2022
Publisher
BioMed Central
Published in
BMC Urology / Issue 1/2022
Electronic ISSN: 1471-2490
DOI
https://doi.org/10.1186/s12894-022-01146-w

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