Collecting duct carcinoma is a rare medullary based high-grade carcinoma

Overlapping features with other medullary based carcinomas make diagnosis challenging

Collecting duct carcinoma lacks recurrent molecular abnormalities

Collecting duct carcinoma is a diagnosis of exclusion

FH-deficient RCC and ALK-RCC have been added to the differential diagnosis

Collecting duct carcinoma of the kidney is a rare and aggressive subtype of renal cell carcinoma (RCC) arising from the distal convoluted tubules. At the time of diagnosis, patients are more frequently symptomatic, with advanced locoregional stage, and have metastatic disease. The 2016 WHO Classification of Tumours of the Urinary System defined diagnostic criteria for this entity. However, the diagnostic features continue to evolve, with typical, but not entirely specific, histologic and immunophenotypic characteristics. In addition, the lack of consistent molecular alterations makes collecting duct carcinoma a diagnosis of exclusion, with historical cases being re-classified as fumarate hydratase deficient RCC, ALK rearranged RCC, renal medullary carcinoma or high-grade urothelial carcinoma. The rarity and poor prognosis of the tumor makes it difficult to reach consensus guidelines to guide therapy. In this manuscript we review the clinicopathologic features of collecting duct carcinoma including pathologic diagnostic criteria, molecular characteristics and differential diagnosis, and their possible implications for management.

Collecting duct carcinoma

Renal cell carcinoma

Non-clear cell renal cell carcinoma

Diagnostic criteria

Differential diagnosis

Clinical management

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