Introduction: Tumor of the renal pelvis was uncommon, as well as the primary mucinous adenocarcinoma of the upper urinary tract. This was aggressive cancer and had a poor prognosis compared with urothelial carcinoma.
Methods: This study presented a 55-year-old male with primary high-grade mucinous adenocarcinoma of the upper urinary tract. From the initial assessment until 8 months of follow up after surgery and adjuvant chemotherapy. A systematic review of the last 20-year-literatures was also performed. There were 32 case reports, with 34 cases reported worldwide.
Results: The majority of the subjects with primary mucinous adenocarcinoma were older male, presented with flank pain, and had a long history of urinary tract stone. The standard surgical modalities were radical nephrectomy, total ureterectomy, and bladder cuff excision. In most cases, only radical nephrectomy was carried out, because the histopathological results were obtained after surgery. The recurrence and distant metastases were widely reported.
Conclusion: The finding of primary mucinous adenocarcinoma of the upper urinary tract was rare and proven after the histopathological finding of the surgical specimen. The treatment modalities were approached with radical nephrectomy, total ureterectomy, followed by chemotherapies with or without radiotherapy.
Key words: mucinous adenocarcinoma, renal pelvis, ureter, nephrectomy