Backgrounds: Ewing Sarcoma (ES) is an aggressive pediatric bone tumor requiring multimodal treatment. Primary renal ES is extremely rare and often presents with nonspecific symptoms, potentially delaying diagnosis. Imaging aids detection, while histopathological and molecular analyses confirm the diagnosis. Case description: We report on a 31-year-old female who was initially treated for pyelonephritis. As computerized tomography suggested a malignant kidney tumor with an extensive inferior vena cava thrombus, nephrectomy and caval ligation were performed. Histopathology and further staging confirmed primary renal ES, which was subsequently treated with adjuvant polychemotherapy. Conclusions: The present case highlights the variable clinical spectrum of malignant kidney tumors. ES should be considered in the differential diagnosis of atypical renal masses.
Lübbersmeyer et al. (2026) studied this question.