EWSR1-fusion-negative, SMARCB1-deficient primary pulmonary myxoid sarcoma
| Autor: | Abbas Agaimy, Thomas Duell, Alicia T. Morresi-Hauf |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: | |
| Zdroj: | Polish Journal of Pathology, Vol 68, Iss 3, Pp 261-267 (2017) |
| Druh dokumentu: | article |
| ISSN: | 1233-9687 2084-9869 |
| DOI: | 10.5114/pjp.2017.71535 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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