Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Renal Clear Cell Sarcoma

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4922-1



Clear cell sarcoma of the kidney is a rare malignant mesenchymal tumor occurring in children.

Clinical Features

  • Incidence

    Clear cell sarcoma comprises 4% of pediatric renal tumors.

  • Age

    It occurs usually at 2–3 years of age and it is extremely rare before 6 months.

  • Sex

    Male-to-female ratio is 1.6:1.

  • Site

    The tumor usually shows the epicenter at the renal medulla.

  • Treatment

    The treatment consists of surgical resection, when possible, followed by chemotherapy and radiotherapy.

  • Outcome

    With current treatment (addition of doxorubicin to chemotherapeutic protocols), outcome has improved with a 5-year overall survival of 75–90%. Bone metastases are common, and unusual sites, such as soft tissues of orbit, may occur. Brain metastases are more common, due to blood-brain barrier which impedes effective chemotherapy. True stage I disease has a favorable prognosis, and chemotherapy protocols are stage-dependent (Argani et al. 2000).


Clear cell sarcoma presents as a well circumscribed, firm, gray mass, frequently located in the renal medulla.


It displays a variable pattern of growth (epithelioid, spindle, sclerosing, myxoid, palisanding), and it is composed by monomorphic small round or spindle or epithelioid cells intermingled with a prominent network of vascular septa (Gooskens et al. 2012) (Fig. 1).
Fig. 1

An epithelioid and spindle pattern of clear cell sarcoma (a). Note the monomorphic small round cells and the vascular network (b)


Vimentin, BCL2, and cyclin D1 are typically positive, whereas CD34, S100, desmin, cytokeratin, and epithelial membrane antigen (EMA) are negative.

Molecular Features

Roughly 10% of clear cell sarcomas harbor the chromosome translocation t(10;17) representing a YWHAE-FAM22 gene fusion, with the remaining cases having other mutation such as BCOR-CCNB3 fusion and EGFR mutations (Aw and Chang 2019).

Differential Diagnosis

Differential diagnosis includes other pediatric small round blue cell tumors, nephroblastoma, and other sarcomas, depending on the prominent patterns above described.

References and Further Reading

  1. Argani, P., Perlman, E. J., Breslow, N. E., et al. (2000). Clear cell sarcoma of the kidney: A review of 351 cases from the National Wilms Tumor Study Group Pathology Center. The American Journal of Surgical Pathology, 24, 4–18.CrossRefGoogle Scholar
  2. Aw SJ, Chang KTE (2019). Clear cell sarcoma of the kidney. Archives of Pathology & Laboratory Medicine. [Epub ahead of print].Google Scholar
  3. Gooskens, S. L., Furtwangler, R., Vujanic, G. M., et al. (2012). Clear cell sarcoma of the kidney: A review. European Journal of Cancer, 48, 2219–2226.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly