Primary pulmonary myxoid sarcoma: A newly described entity - Report of a case and review of the literature Journal Article
| Authors: | Prieto-Granada, C. N.; Ganim, R. B.; Zhang, L.; Antonescu, C.; Mueller, J. |
| Article Title: | Primary pulmonary myxoid sarcoma: A newly described entity - Report of a case and review of the literature |
| Abstract: | Primary pulmonary myxoid sarcoma (PPMS) is a recently described, exceedingly rare low-grade lung sarcoma that tends to present in young females as an endobronchial mass and shows evidence of an EWSR1-CREB1 fusion. Herein, we present a case of PPMS with fluorescence in situ hybridization (FISH) analysis for EWSR1 and CREB1 rearrangements. An 80-year-old woman presented with an endobronchial, multinodular tumor exhibiting spindle, ovoid and epithelioid cells arranged in reticular/lattice-like and alveolar-like patterns in a myxoid background. The tumor showed focal epithelial membrane antigen immunoreactivity as well as an Alcian blue-positive stroma that was sensitive to digestion with hyaluronidase. EWSR1 and CREB1 rearrangements were detected by break-apart FISH probes. The patient showed persistence of disease 36 months after diagnosis and was discharged to hospice care. We contribute with a report of an additional case of this very unusual entity and perform a brief review of the literature published so far on the subject. © The Author(s) 2017. |
| Keywords: | fluorescence in situ hybridization; lung sarcoma; ewsr1-creb1; primary pulmonary myxoid sarcoma |
| Journal Title: | International Journal of Surgical Pathology |
| Volume: | 25 |
| Issue: | 6 |
| ISSN: | 1066-8969 |
| Publisher: | Sage Publications |
| Date Published: | 2017-09-01 |
| Start Page: | 518 |
| End Page: | 525 |
| Language: | English |
| DOI: | 10.1177/1066896917706413 |
| PROVIDER: | scopus |
| PUBMED: | 28449608 |
| DOI/URL: | |
| Notes: | Review -- Export Date: 5 September 2017 -- Source: Scopus |
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