Clear-cell sarcoma of the kidney

Clear-cell sarcoma of the kidney
Classification and external resources
ICD-O 8964/3

Clear cell sarcoma of the kidney (CCSK) is an extremely rare type of kidney cancer comprising 3% of all pediatric renal tumours.[1] Clear cell sarcoma of the kidney can spread from the kidney to other organs, most commonly the bone, but also including the lungs, brain, and soft tissues of the body.

Despite the similarities in names, clear cell sarcoma of the kidney is unrelated to clear cell sarcoma of soft tissue, also known as malignant melanoma of soft parts.[2]

Etiology and epidemiology

Research in 2005 showed that CCSK can arise within a renal mesenchymal cell that shows a wide variety of neural markers.[3] Typical presentation is between 1 and 4 years of age, and a 2:1 male-to-female ratio is observed.[4]

Clinical presentation

Typical gross features include large size (mean diameter 11.3 cm), a mucoid texture, foci of necrosis, and prominent cyst formation.[5]


2004 research showed that CCSK patients exhibit an improved relapse-free survival from a longer course of therapy when using vincristine, doxorubicin, and dactinomycin, but their long-term survival is unchanged compared with patients receiving 6 months of therapy.[6]


  1. Sharma SC, Menon PA, Clear cell sarcoma of the kidney, Journal of Postgraduate Medicine, 2001, vol. 47, p. 206, PMID 11832627. Accessed 2012-5-21.
  2. Poppe B, Forsyth R, Dhaene K, Speleman F., Soft tissue tumors: Clear cell sarcoma, Atlas Genet Cytogenet Oncol Haematol, November 2002.
  3. Cutcliffe C, Kersey D, Huang CC, Zeng Y, Walterhouse D, Perlman EJ, Clear Cell Sarcoma of the Kidney: Up-regulation of Neural Markers with Activation of the Sonic Hedgehog and Akt Pathways, Clinical Cancer Research, Nov 15 2005, 11(22), pp. 7986-94, doi:10.1158/1078-0432.CCR-05-1354, PMID 16299227. Accessed 2012-5-21.
  4. Seibel N, Clear Cell Sarcoma of the Kidney, Medscape Reference, Updated 2014-2-11, Accessed 2014-5-18.
  5. Argani, Pedram M.D.; Perlman, Elizabeth J. M.D.; Breslow, Norman E. Ph.D.; Browning, Nancy G. M.S.; Green, Daniel M. M.D.; D'Angio, Giulio J. M.D.; Beckwith, J. Bruce M.D., Clear Cell Sarcoma of the Kidney: A Review of 351 Cases From the National Wilms Tumor Study Group Pathology Center, American Journal of Surgical Pathology, Jan 2000, 24(1), p. 4, Accessed 2012-5-21.
  6. Effect of Duration of Treatment on Treatment Outcome for Patients With Clear-Cell Sarcoma of the Kidney: A Report From the National Wilms' Tumor Study Group, Journal of Clinical Oncology, Feb 1 2004, vol. 22(3), pp. 468-473, doi:10.1200/JCO.2004.06.058. Accessed 2012-5-21.

 This article incorporates public domain material from the U.S. National Cancer Institute document "Dictionary of Cancer Terms".

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