Clear-cell sarcoma of the kidney explained
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]
Cause
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]
Treatment
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]
External links
Notes and References
- Sharma SC, Menon PA . Clear cell sarcoma of the kidney . Journal of Postgraduate Medicine . 47 . 3 . 206–7 . 2001 . 11832627 .
- Poppe B, Forsyth R, Dhaene K, Speleman F . Soft tissue tumors: Clear cell sarcoma. . Atlas of Genetics and Cytogenetics in Oncology and Haematology . 2003 . 7 . 1 . 49–53 . 10.4267/2042/37939 . free . 1854/LU-4181590 . free .
- Argani P, Perlman EJ, Breslow NE, Browning NG, Green DM, D'Angio GJ, Beckwith JB . 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 . 1 . 4–18 . January 2000 . 10632483 . 10.1097/00000478-200001000-00002 . free .
- 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 . 11 . 22 . 7986–94 . November 2005 . 16299227 . 10.1158/1078-0432.CCR-05-1354 . 9130030 . free .
- Web site: Seibel N . Clear Cell Sarcoma of the Kidney . Medscape Reference . 11 February 2014 . 18 May 2014 .
- Seibel NL, Li S, Breslow NE, Beckwith JB, Green DM, Haase GM, Ritchey ML, Thomas PR, Grundy PE, Finklestein JZ, Kim T, Shochat SJ, Kelalis PP, D'Angio GJ . 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 . 22 . 3 . 468–73 . February 2004 . 14752069 . 10.1200/JCO.2004.06.058 . free .
