High grade infiltrative adenocarcinomas of renal cell origin: New insights into classification, morphology, and molecular pathogenesis

Pathol Int. 2018 May;68(5):265-277. doi: 10.1111/pin.12667. Epub 2018 Apr 17.


Collecting duct carcinoma was described over 30 years ago as a renal tumor, based in the medullary collecting system, with tubulopapillary morphology, prominent infiltrative growth, and stromal desmoplasia. While diagnostic workup has always emphasized exclusion of upper tract urothelial carcinoma and metastatic adenocarcinoma to the kidney, the molecular era of renal cell carcinoma classification has enabled recognition of and provided tools for diagnosis of new entities in this morphologic differential. In this review, we consider these developments, with emphasis on renal medullary carcinoma, closely related renal cell carcinoma, unclassified with medullary phenotype, and fumarate hydratase-deficient renal cell carcinoma. Integration of ancillary studies with suggestive patterns of morphology is emphasized for practical implementation in contemporary diagnosis, and several emerging tumor types in the morphologic differential are presented.

Keywords: collecting duct carcinoma; fumarate hydratase-deficient renal cell carcinoma; renal cell carcinoma; renal medullary carcinoma; unclassified; with medullary phenotype.

Publication types

  • Review

MeSH terms

  • Carcinoma, Renal Cell* / classification
  • Carcinoma, Renal Cell* / genetics
  • Carcinoma, Renal Cell* / pathology
  • Humans
  • Kidney Neoplasms* / classification
  • Kidney Neoplasms* / genetics
  • Kidney Neoplasms* / pathology