6^th European Pathology Congress

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.They are defined by distinctive histomorphological features and characterised by a recurrent fusion gene. All tumours involved pulmonary parenchyma with a predilection for the endobronchial component. They appear to have a 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 within myxoid stroma, morphologically reminiscent of extra skeletal 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 sarcoma – like tumours of the gastrointestinal tract and angiomatoid fibrous histiocytomas, however this is a novel finding in tumours with the morphology described and occurring in the pulmonary region.