Clear-cell sarcoma of the kidney

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Clear-cell sarcoma of the kidney
Gross pathological findings. The tumor weighed approximately 750 g and measured 15 × 12 × 9 cm. The left kidney is replaced by the neoplasm. Calcification is apparent around the necrotic tissue
SpecialtyOncology, nephrology
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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]

Signs and symptoms

Typical gross features include large size (mean diameter 11.3 cm), a mucoid texture, foci of necrosis, and prominent cyst formation.[3]


Research in 2005 showed that CCSK can arise within a renal mesenchymal cell that shows a wide variety of neural markers.[4] Typical presentation is between 1 and 4 years of age, and a 2:1 male-to-female ratio is observed.[5]


Differential diagnosis

The DDX for this condition is based on the following:[6]

  • Cellular congenital mesoblastic nephroma
  • Blastemal WT
  • Metanephric stromal tumor


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.[7]


  1. Sharma SC, Menon PA, Clear cell sarcoma of the kidney Archived 2021-04-10 at the Wayback Machine, 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 Archived 2010-07-03 at the Wayback Machine, Atlas Genet Cytogenet Oncol Haematol, November 2002.
  3. 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 Archived 2017-03-13 at the Wayback Machine, American Journal of Surgical Pathology, Jan 2000, 24(1), p. 4, Accessed 2012-5-21.
  4. 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 Archived 2012-07-13 at, 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.
  5. Seibel N, Clear Cell Sarcoma of the Kidney Archived 2021-02-26 at the Wayback Machine, Medscape Reference, Updated 2014-2-11, Accessed 2014-5-18.
  6. Aldera, Alessandro Pietro; Pillay, Komala (27 March 2019). "Clear Cell Sarcoma of the Kidney". Archives of Pathology & Laboratory Medicine. 144 (1): 119–123. doi:10.5858/arpa.2018-0353-RS. ISSN 0003-9985. Archived from the original on 27 February 2021. Retrieved 16 September 2021.
  7. 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 Archived 2021-09-23 at the Wayback Machine, Journal of Clinical Oncology, Feb 1 2004, vol. 22(3), pp. 468-473, doi:10.1200/JCO.2004.06.058. Accessed 2012-5-21.

External links

External resources

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