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

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.

Unveiling the Uncommon: A Case of Metastatic Ewing Sarcoma of the Kidney

“…ESK frequently spreads to various locations in approximately 65% of patients, with the lungs, liver, and regional lymph nodes being the most commonly affected sites [ 4 ]. Our patient had metastases to the lungs.…”

“…ESK is an exceedingly rare and highly malignant entity, first reported by Seemayer et al in 1975 and approximately comprising less than 200 cases in the literature [12][13][14]. These tumors, which make up <1% of all renal tumors, have a much higher prevalence in males than females [4]. A meta-analysis done by Risi et al of 116 cases of ESK found that the median age was 28 years old and the most common presenting clinical symptoms included pain (54%), hematuria (29%), and renal mass (28%) [15].…”

“…It is most commonly diagnosed in young adults and adolescents with a peak incidence at 15 years of age [ 1 ]. The occurrence of Ewing sarcoma of the kidney (ESK) is rare, comprising only 1% of all renal tumors [ 4 ]. Patients generally present with a mass in the abdomen or with renal colic symptoms like abdominal pain and hematuria [ 4 ].…”

“…The occurrence of Ewing sarcoma of the kidney (ESK) is rare, comprising only 1% of all renal tumors [ 4 ]. Patients generally present with a mass in the abdomen or with renal colic symptoms like abdominal pain and hematuria [ 4 ]. Subsequently, due to its occult intra-abdominal location, the tumor usually presents as a sizeable mass before it is detected [ 2 ].…”

“…Subsequently, due to its occult intra-abdominal location, the tumor usually presents as a sizeable mass before it is detected [ 2 ]. Given the aggressive nature of this tumor, metastasis occurs in 65% of cases, frequently involving the lungs but can also spread to regional lymph nodes and the liver [ 4 ].…”