Biography
Biography: Shroque Zaher
Abstract
This case represents a rare entity- primary pulmonary myxoid sarcoma, of which to the best of our knowledge only 10 other cases have been reported in the literature. (Thway et al, 2011). They are defined by distinctive histomorphological features and characterized by a recurrent fusion gene. All tumours involved pulmonary parenchyma with a predilection for the endobronchial component. They appear to have a predilection for females, with 7 of the 10 reported cases , occurring in women. Microscopically, they are lobulated tumours comprising cords of polygonal, spindle, stellate cells with myxoid stroma, morphologically reminiscent of extraskeletal myxoid chondrosarcoma. Tumours were immunoreactive for only vimentin and weakly focal for EMA, although our specific case was negative for these markers. In 7 of the 10 tumours, a specific EWSR1-CREB1 fusion gene was demonstrated by reverse transcription-polymerase chain reaction. This gene fusion has been described previously in 2 histologically and behaviorally different sarcomas: clear cell like sarcoma-like tumours of the gastrointestinal tract and angiomatoid fibrous histiocytoma, however this is a novel finding in tumours with the morphology described and occurring in the pulmonary region.