Clear cell sarcoma of kidney

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Synonyms: CCSK

Clear cell sarcoma of kidney is a rare primary genetic renal tumor usually characterized by a unilateral unicentric morphologically diverse tumor that arises from the renal medulla and has a tendency for vascular invasion. Clinically it presents with a palpable abdominal mass abdominal or flank pain hematuria anemia and/or fatigue. Metastatic spread to lymph nodes bones lungs retroperitoneum brain and liver is common at time of diagnosis and therefore bone pain cough or neurological compromise may be associated. Metastasis to unusual sites such as the scalp neck nasopharynx axilla orbits and epidural space have been reported.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version July 2024

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