Primary palatal sarcoma exhibiting EWSR1::RORß fusion: a first case report and literature review.

In this report, a tumor exhibited EWSR1 :: RORß gene fusion, to our knowledge, is the first such reported case. The Ewing sarcoma breakpoint region 1 gene (EWSR1) is known to be associated with several soft tissue tumors although its specific role remains unclear. Its fusion with a member of the ETS family, including FLI1 and ERG , results in Ewing sarcoma, and its fusion with other genes unrelated to the ETS family, including NFATC2 and PATZ1 , results in round cell sarcoma with EWSR1-non-ETS fusions, previously referred to as Ewing-like sarcoma. RORß encodes retinoic acid-related orphan receptor ß, a nuclear receptor (NR), and is involved in circadian rhythm modulation and cancer regulation. The specific role of ROR ß in tumorigenesis remains unclear; however, this case report suggests that it may form part of a new tumorigenic entity. [ABSTRACT FROM AUTHOR]