Clear Cell Sarcoma of the Kidney

Arch Pathol Lab Med. 2019 Aug;143(8):1022-1026. doi: 10.5858/arpa.2018-0045-RS. Epub 2019 Jan 10.


Clear cell sarcoma of the kidney is the second most common primary renal malignancy in childhood. It is histologically diverse, making accurate diagnosis challenging in some cases. Recent molecular studies have uncovered BCOR exon 15 internal tandem duplications in most cases, and YWHAE-NUTM2 fusion in a few cases, with the remaining cases having other genetic mutations, including BCOR-CCNB3 fusion and EGFR mutations. Although clear cell sarcoma of the kidney has no specific immunophenotype, several markers including cyclin D1, nerve growth factor receptor, and BCOR (BCL6 corepressor) have emerged as potential diagnostic aides. This review provides a concise account of recent advances in our understanding of clear cell sarcoma of the kidney to serve as a practical update for the practicing pathologist.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism*
  • Child
  • Cyclin B / genetics
  • Cyclin B / metabolism
  • Diagnosis, Differential
  • Humans
  • Kidney / metabolism*
  • Kidney / pathology
  • Kidney Neoplasms / diagnosis
  • Kidney Neoplasms / genetics
  • Kidney Neoplasms / metabolism*
  • Mutation
  • Proto-Oncogene Proteins / genetics
  • Proto-Oncogene Proteins / metabolism
  • Repressor Proteins / genetics
  • Repressor Proteins / metabolism
  • Sarcoma, Clear Cell / diagnosis
  • Sarcoma, Clear Cell / genetics
  • Sarcoma, Clear Cell / metabolism*


  • BCOR protein, human
  • Biomarkers, Tumor
  • CCNB3 protein, human
  • Cyclin B
  • Proto-Oncogene Proteins
  • Repressor Proteins