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  • Title: EWSR1-fusion-negative, SMARCB1-deficient primary pulmonary myxoid sarcoma.
    Author: Agaimy A, Duell T, Morresi-Hauf AT.
    Journal: Pol J Pathol; 2017; 68(3):261-267. PubMed ID: 29363919.
    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.
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