Clear-cell sarcoma of the kidney

Clear cell sarcoma of the kidney (CCSK) is a rare type of kidney cancer comprising 4% of all primary renal tumours. Clear cell sarcoma 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.

Etiology and epidemiology
Research in 2005 showed that CCSK can arise within a renal mesenchymal cell that shows a wide variety of neural markers.

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

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.