Pathological Advances and Clinical Management Insights into Primary Pulmonary Myxoid Sarcoma Harboring the Rare EWSR1 :: CREB1 Fusion

Primary pulmonary myxoid sarcoma (PPMS) is an extremely rare malignant pulmonary neoplasm. Its pathogenesis is closely linked to the chromosomal translocation t(2;22)(q33;q12), which drives the formation of the EWSR1::CREB1 gene fusion. This pathognomonic genetic alteration serves as one of the key clues for the pathological diagnosis of PPMS. In this paper, we report a patient with PPMS, a 64-year-old female patient, in whom the EWSR1::CREB1 gene fusion was definitively confirmed via fluorescence in situ hybridization. The tumor had been present for 7 years prior to resection without causing symptoms, suggesting an indolent growth pattern. The patient received no adjuvant therapy and remained without evidence of disease at an interval of 46 months following surgery. The main value of this report is an additional patient with a detailed documentation of indolent behavior, both pre-operatively and post-operatively.