Section Editor: Dharam M. Ramnani, MD

Virginia Urology, Richmond, VA, USA

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Clear Cell Sarcoma of Kidney (CCSK) - Metastasis: The most frequent site of metastasis at presentation is the ipsilateral renal hilar lymph nodes (about 30% of cases). This is due to the ability of tumor cells to invade renal and perirenal lymphovascular system. Some cases with negative renal hilar lymph nodes may show skip metastases to periaortic lymph nodes.

CCSK has a propensity for late recurrences, especially involving bone (hence the previous name - bone metastasizing renal tumor of childhood) and lungs. Other sites include retroperitoneum, brain and liver. In contrast, Wilms tumor spreads to lymph nodes, lung, and liver but rarely involves bones. Uncommonly, CCSK can metastasize to unusual sites, including scalp, epidural, nasopharynx, neck, paraspinal, soft tissues, abdominal wall, axilla, and orbit.

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