Medicine:Clear-cell sarcoma of the kidney
| Clear-cell sarcoma of the kidney | |
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| Specialty | Oncology, nephrology |
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]
References
- ↑ "Clear cell sarcoma of the kidney". Journal of Postgraduate Medicine 47 (3): 206–7. 2001. PMID 11832627.
- ↑ "Soft tissue tumors: Clear cell sarcoma.". Atlas of Genetics and Cytogenetics in Oncology and Haematology 7 (1): 49–53. 2003. doi:10.4267/2042/37939. https://biblio.ugent.be/publication/4181590.
- ↑ "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. doi:10.1097/00000478-200001000-00002. PMID 10632483.
- ↑ "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. doi:10.1158/1078-0432.CCR-05-1354. PMID 16299227.
- ↑ "Clear Cell Sarcoma of the Kidney". Medscape Reference. 11 February 2014. http://emedicine.medscape.com/article/993245-overview.
- ↑ "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. doi:10.1200/JCO.2004.06.058. PMID 14752069.
External links
- Clear cell sarcoma of the kidney entry in the public domain NCI Dictionary of Cancer Terms
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| External resources |
This article incorporates public domain material from the U.S. National Cancer Institute document "Dictionary of Cancer Terms".
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