Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain origin, exhibiting a wide clinical and morphological spectrum. It ranges from benign forms, which typically behave indolently, to malignant lesions with significant recurrence and metastatic potential. The majority of OFMTs harbor PHF1 gene rearrangements, with EP400 being the most common fusion partner. Herein, we present a patient with malignant metastatic OFMT, with the very rare PHF1::FOXR2 fusion, and discuss the potential clinical implications of this genetic alteration.
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