Primary Pulmonary Myxoid Sarcoma And Myxoid Angiomatoid Fibrous Histiocytoma A Unifying Continuum With Shared And Distinct Features

Primary pulmonary myxoid sarcoma (PPMS) is a recently reported, exceedingly rare low-grade lung neoplasm characterized by reticular/lace-like growth of spindle to epithelioid cells embedded in an abundant myxoid matrix. Morphologically, it overlaps with a myxoid variant of angiomatoid fibrous histiocytoma (AFH) of the soft tissue. Genetically, they were both reported to harborEWSR1-CREB1fusion, whileEWSR1-ATF1has only been reported in AFH thus far. We report a case of primary pulmonary low-grade myxoid spindle cell tumor with morphologic and immunohistochemical features of PPMS but with anEWSR1-ATF1fusion gene. In addition, we also encountered a case of endobronchial AFH withEWSR1-CREB1translocation but also focal morphologic features of PPMS. These findings provide new evidence supporting the concept that PPMS and a myxoid variant of AFH represent a continuum with overlapping histologic, immunohistochemical, and genetic features.