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However, the patient's symptoms did not improve. Consequently, right nephrectomy was performed for both diagnosis and treatment, which revealed a sarcomatoid RCC with metastasis to the regional lymph node. The patient gradually developed cachexia and died on day 106 post-admission. The present case illustrates the difficulty of diagnosing RCC in patients with ADPKD, particularly sarcomatoid RCC, which is a rare and aggressive variant of RCC, even with the use of various types of imaging modalities. An early decision of nephrectomy may be