According to previous literature, intra-abdominal epithelioid neoplasms with EWSR1::CREB fusions originating from the kidney should be considered in the differential diagnosis of sclerosing epithelioid fibrosarcoma (SEF), angiomatoid fibrous histiocytoma (AFH), epithelioid mesothelioma (EM), clear cell sarcoma of the kidney (KCCS), synovial sarcoma (SS), solitary fibrous tumors (SFT), or metanephric stromal tumor (MST) (4). This evidence concerns the gene CREB1 and transient myeloproliferative syndrome.