{Reference Type}: Case Reports
{Title}: EWSR1-fusion-negative, SMARCB1-deficient primary pulmonary myxoid sarcoma.
{Author}: Agaimy A;Duell T;Morresi-Hauf AT;
{Journal}: Pol J Pathol
{Volume}: 68
{Issue}: 3
{Year}: 2017
{Factor}: 0.909
{DOI}: 10.5114/pjp.2017.71535
{Abstract}: 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.