Abstract
BACKGROUND: Upper tract urothelial carcinoma with pure non-urothelial histology is an exception but variants are present in ~ 25% of cases. Primary upper urinary tract signet -ring cell carcinoma is extremely rare. CASE PRESENTATION: We report the case of a 65-year-old male diagnosed primary upper urinary tract signet-ring cell carcinoma while underwent percutaneous nephrolithotomy. Radical nephroureterectomy and adjuvant chemotherapy were performed sequentially. The patient is now recovering well with a regular follow-up for more than 1 year. CONCLUSIONS: The upper urinary tract malignancy often appears as a high grade, high stage tumor and has a uniformly poor prognosis, but a timely multimodal management can bring a good outcome.