Abstract
Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumors typically found in children and adolescents. These tumors belong to the family of small round cell tumors and are of neuroectodermal origin. Primary Ewing sarcoma of the kidney is rare and because of that is an infrequent differential diagnosis in urologic malignancies. Renal PNET mostly presents with nonspecific symptoms such as hematuria and abdominal pain. The imaging findings are uncharacteristic. The diagnosis is based on the histology, immunohistochemistry, and molecular pathologic findings. Once PNET has been diagnosed, multimodal treatment is indicated. Despite all treatment options, the prognosis of those with metastatic disease is poor.
Highlights
Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, both share common stem-cell precursor and unique chromosomal abnormality
Renal cell carcinoma (RCC) is the most common malignant neoplasm of the kidney that constitutes more than 90% of
No specific signs of PNET have been described in ultrasonography, computed tomography (CT), or magnetic resonance imaging (MRI)
Summary
Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are considered part of a spectrum of neoplastic diseases known as Ewing’s sarcoma family tumors (ESFT), which includes other malignancies [1,2,3]. Due to worsening of the symptoms, an abdominal ultrasound was performed, which showed an infiltrative mass in the upper pole of the right kidney, invading the right liver, concerning for malignancy (Figure 1). Biopsy of the kidney mass revealed Ewing sarcoma/primitive neuroectodermal tumor (Figure 5).
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