%0 Journal Article %J Polish Journal of Pathology %@ 1233-9687 %V 68 %N 3 %D 2017 %F Agaimy2017 %T EWSR1-fusion-negative, SMARCB1-deficient primary pulmonary myxoid sarcoma %X Primary pulmonary myxoid sarcoma (PPMS) is a recently defined rare neoplasm with histological and molecular similarity to extraskeletal myxoid chondrosarcoma. To date, 20 cases have been reported. A 48-year-old man presented with a huge mass filling the right hemithorax and extending into the tracheobronchial system. Histological findings were consistent with PPMS. Immunohistochemistry was positive for vimentin, CD10, and EMA, but other lineage-specific markers were negative. SMARCB1 (INI1) expression was lost in the tumour cells. FISH analysis ( EWSR1 , FUS , NR4A3 , and SMARCB1 ) revealed no abnormalities. This case suggests SMARCB1 loss as a possible alternative molecular event driving EWSR1-negative PPMS. %A Agaimy, Abbas %A Duell, Thomas %A Morresi-Hauf, Alicia T. %P 261-267 %9 journal article %R 10.5114/pjp.2017.71535 %U http://dx.doi.org/10.5114/pjp.2017.71535