Clear Cell Sarcoma of the Kidney
Also written as: CCSK
A malignant paediatric renal tumour characterised by a branching vascular pattern and bland cells with pale nuclei, notorious for late skeletal metastases and not related to clear cell sarcoma of soft tissue.
Full Definition
Clear cell sarcoma of the kidney (CCSK) is the second most common malignant renal tumour in children after Wilms tumour. Its name refers to the cytological appearance of the tumour cells rather than to a relationship with soft-tissue clear cell sarcoma. The classic pattern features cords and nests of cells with pale cytoplasm separated by a delicate arborising vasculature. CCSK has a propensity for bone metastases, distinguishing it clinically from Wilms tumour. Editors must be alert to the misleading overlap in terminology with soft-tissue clear cell sarcoma and should ensure the organ of origin is always stated.
Usage
Usage note: Always specify 'of the kidney'; unqualified 'clear cell sarcoma' defaults to the soft-tissue entity in general oncology contexts.
In Context
- "The pathology report described classic clear cell sarcoma of the kidney with the hallmark arborising vascular pattern." — Paediatric nephrology case report
- "When editing reports, confirm that 'clear cell sarcoma' is qualified by site, as the soft-tissue counterpart is a distinct entity." — Editorial annotation