Kidney tumor
Adult renal cell carcinoma
Cystic renal epithelial neoplasms

Topic Completed: 1 July 2016

Revised: 7 March 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Cystic renal epithelial neoplasms

Nicole K. Andeen, M.D.
Maria Tretiakova, M.D., Ph.D.
Page views in 2018: 2,276
Page views in 2019 to date: 1,676
Table of Contents
Definition / general
Cite this page: Andeen NK, Tretiakova M. Cystic renal epithelial neoplasms. website. Accessed July 20th, 2019.
Definition / general

Multilocular cystic renal neoplasm of low malignant potential
  • "Neoplasm composed entirely of numerous cysts, the septa of which contain individual or groups of clear cells without expansile growth" (WHO 2016) clear cells have low grade nuclei (ISUP 1 or 2)
  • Multicystic with no expansile growth / solid nodules

Cystic clear cell papillary renal cell carcinoma
  • Linear arrangement of nuclei away from basal membrane
  • Coexpression of CK7 & CAIX (cup shaped positivity)

Clear cell renal cell carcinoma with cystic degeneration
  • An extensively cystic / necrotic tumor may still exhibit aggressive behavior even if only rare identifiable tumor cells (Am J Surg Pathol 2000;24:988)
  • Hemorrhage, necrosis, thick irregular cyst wall, may be papillary / pseudopapillary, may have large areas of solid architecture or few residual tumor cells.

Clear cell renal cell carcinoma originating in a benign cyst
Acquired cystic disease associated renal cell carcinoma
  • Most common kidney tumor in patients with end stage renal disease and acquired cystic disease
  • Characterized by abundant calcium oxalate crystals and variable morphology: papillary, solid, eosinophilic and clear cell-like areas

Tubulocystic renal cell carcinoma
  • Sponge-like, composed of small to intermediate size cysts
  • Usually enlarged nuclei and ISUP grade 3 nucleoli
  • Shares cytogenetic abnormalities with papillary RCC (trisomy 7 / 17).

Cystic nephroma (in adult)
  • Multiloculated benign tumor with cysts lined by flat to hobnailed epithelial cells separated by fibrous septa containing paucicellular or ovarian-like stroma; no septal nodules of clear cells
  • Part of a spectrum of renal epithelial and stromal tumors that includes mixed epithelial and stromal tumor (Am J Surg Pathol 2009;33:72, Am J Surg Pathol 2007;31:489)
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