Abstract
BACKGROUND: Renal pelvis sarcomatoid carcinoma (RPSC) is a rare and aggressive malignancy whose diagnosis is difficult because radiological imaging results can lead to misclassification as a more common type of renal tumor. In addition, clinical management of patients with RPSC is difficult because of the limited efficacy of available treatments. In this study, we present a comprehensive description of a patient who presented with RPSC and a simultaneous renal vein tumor thrombus. CASE SUMMARY: During April, 2020, a 64-year-old female presented with an isolated episode of hematuria accompanied by abdominal pain. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a lesion in the right renal pelvis. We therefore performed a radical nephrectomy of the right kidney. The subsequent histopathological and immunological results verified the diagnosis of RPSC. Despite administration of 6 cycles of a gemcitabine-cisplatin regimen, the patient's condition progressively deteriorated, and she died about 15 mo after the nephrectomy. CONCLUSION: We performed a comprehensive analysis of a patient with RPSC that included CT, MRI, immunohistochemistry, and genetic testing. The insights from our detailed analysis of this patient and our concomitant review of the literature may assist clinicians in their diagnosis and treatment of RPSC.