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Clear cell sarcoma is a rare form of cancer called a sarcoma. It is known to occur mainly in the soft tissues and dermis. Rare forms were thought to occur in the gastrointestinal tract before they were discovered to be different and redesignated as GNET.
Recurrence is common.
Clear cell sarcoma's neoplastic cells express the EWSR1-ATF1 fusion gene in a majority of cases or a EWSR1-CREB1, EWSR1-CREM, or EWSR1-DDIT3 fusion gene in a small subset of cases.
Clear cell sarcoma of the soft tissues in adults is not related to the pediatric tumor known as clear cell sarcoma of the kidney.
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