Findings demonstrate the highly aggressive nature of collecting duct carcinoma (CDC) of the kidney, and diagnosis at an advanced stage is common. An improved prognosis can be offered by early surgical treatment. Though standard treatment is lacking, immune checkpoint inhibitors (ICIs) bring new hope for the treatment of CDC.

• In this analysis of 74 patients with CDC (mean age was 61.5 years, 54.1% were males), the most common symptoms included low back pain, hematuria, and fatigue.

• Lack of specificity of computed tomography was evident, with 10.8% of the cases diagnosed with urothelial carcinoma and 4.1% with infectious disease.

• Presence of metastasis at presentation in 32 patients, and tumor thrombus in the venous system in 17.6%, was documented.

• Renal biopsy was performed in 22 patients, and CDC was detected in 50% of the patients.

• Overall 61 renal surgeries were conducted, and 8 patients were treated with ICIs, and an objective remission rate of 50% was obtained.

• Median overall survival was 24.0 months, and sarcomatoid differentiation and absence of renal surgery were identified as predictors of mortality.