Among these, EWSR1::CREB1/ATF1/CREM fusions have been recognized as the most prevalent drivers in clear cell sarcoma of tendons and aponeuroses (CCS) [9], malignant gastrointestinal neuroectodermal tumor (MGNET) [10], angiomatoid fibrous histiocytoma (AFH) [11–13], primary pulmonary myxoid sarcoma [14] as well as in emerging categories of epithelioid, mostly keratin-positive intra-abdominal neoplasms/sarcomas [15] and in primary intracranial myxoid sarcomas not fitting any specific category [16]. The gene discussed is CREM; the disease is histiocytoma, Angiomatoid fibrous.