Clear Cell Papillary Cystadenoma of the Ovary Masquerading as Metastatic Clear Cell Renal Cell Carcinoma: A Case Report and Review of the Literature

Int J Gynecol Pathol. 2021 May 1;40(3):290-295. doi: 10.1097/PGP.0000000000000666.


Clear cell papillary cystadenoma of the epididymis is an uncommon benign neoplasm, usually seen in patients with von Hippel-Lindau disease. Morphologic and immunohistochemical examination aid in distinguishing clear cell papillary cystadenoma from malignant histologic mimics including low-grade mesothelial proliferations and metastatic clear cell renal cell carcinomas. Analogous lesions have been described in the female genital tract, often posing diagnostic challenges due to their low incidence. Here, we present the difficult diagnostic aspects of the first case of clear cell papillary cystadenoma involving the ovary, including the salient immunohistochemical, ultrastructural, and molecular characteristics.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Carcinoma, Renal Cell / diagnostic imaging*
  • Carcinoma, Renal Cell / pathology
  • Cystadenoma, Papillary / diagnostic imaging*
  • Cystadenoma, Papillary / genetics
  • Cystadenoma, Papillary / pathology
  • Diagnosis, Differential
  • Female
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Immunohistochemistry
  • Microscopy, Electron
  • Middle Aged
  • Ovarian Neoplasms / diagnostic imaging*
  • Ovarian Neoplasms / pathology
  • Ovary / diagnostic imaging
  • Ovary / pathology
  • Point Mutation
  • Sequence Analysis, DNA
  • Von Hippel-Lindau Tumor Suppressor Protein / genetics


  • Von Hippel-Lindau Tumor Suppressor Protein
  • VHL protein, human

Supplementary concepts

  • Clear-cell metastatic renal cell carcinoma