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Staging of Clear Cell Sarcoma of Kidney (CCSK): The staging is done according to National Wilms Tumor Study protocol as follows:

Stage 1: Tumor confined to the kidney and completely resected. No penetration of the renal capsule or involvement of renal sinus vessels. About 25% of patients present at Stage 1.

Stage 2: Tumor extends beyond kidney but completely resected. Tumor penetrates renal capsule, invades sinus vessels, was biopsied before removal, or spilled locally during removal, but margins are negative. About 37% of patients have Stage 2 tumors.

Stage 3: Gross residual tumor, positive surgical margins, massive tumor spill or lymph node metastases. About 34% of patients have Stage 3 tumors at diagnosis.

Stage 4: Hematogenous metastases. About 4% of patients have Stage 4 disease.

Stage 5: Bilateral renal tumors. Rare cases present at Stage 5.

This image of classic pattern of CCSK shows nests of small uniform polygonal to ovoid to spindle cells, separated by regularly spaced vascular channels.

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