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.
Urologic oncology. 2021 Jun 08 [Epub ahead of print]
Gerardo Cabanillas, Diego Montoya-Cerrillo, Oleksandr N Kryvenko, Sumanta K Pal, Javier A Arias-Stella
Internal Medicine Department, Pacifica Hospital of the Valley, Serra Medical Group, Sun Valley, CA., Department of Pathology and Laboratory Medicine., Department of Pathology and Laboratory Medicine; Department of Urology; Sylvester Comprehensive Cancer Center, University of Miami / Jackson Memoria Hospital, Miami, FL., Department of Medical Oncology & Experimental Therapeutics, City of Hope National Medical Center, Duarte, CA., Department of Pathology, City of Hope National Medical Center, Duarte, CA. Electronic address: .