Abstract
BACKGROUND: Primary squamous cell carcinoma (SCC) with sarcomatoid differentiation of the kidney was rarely reported. This disease is usually related to renal stones, and due to a lack of symptoms and radiological features, patients usually attend the hospital with late stage disease. CASE SUMMARY: A 54-years-old female presented with left flank pain and an abdominal mass for 6 mo. Imaging studies revealed that the left kidney was enlarged and massive hydronephrosis was present. A stone was seen in the ureteropelvic junction. The patient subsequently underwent left radical nephrectomy, and histopathological examination of the mass revealed a poorly differentiated renal SCC with sarcomatoid differentiation. After primary surgery, the patient received four cycles of tirelizumab. Four months later, the patient developed adrenal, lymph, and uterine appendage metastases. CONCLUSION: SCC of the kidney has a poor prognosis, and should be considered in patients with a renal mass, long-standing urinary calculi and massive hydronephrosis.