{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T10:48:14Z","timestamp":1782211694844,"version":"3.54.5"},"reference-count":16,"publisher":"Termedia Sp. z.o.o.","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pol J Pathol"],"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 (<i>EWSR1<\/i>, <i>FUS<\/i>, <i>NR4A3<\/i>, and <i>SMARCB1<\/i>) revealed no abnormalities. This case suggests SMARCB1 loss as a possible alternative molecular event driving EWSR1-negative PPMS.<\/jats:p>","DOI":"10.5114\/pjp.2017.71535","type":"journal-article","created":{"date-parts":[[2018,3,8]],"date-time":"2018-03-08T04:51:06Z","timestamp":1520484666000},"page":"261-267","source":"Crossref","is-referenced-by-count":14,"title":["EWSR1-fusion-negative, SMARCB1-deficient primary pulmonary myxoid sarcoma"],"prefix":"10.5114","volume":"68","author":[{"given":"Abbas","family":"Agaimy","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Duell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia T.","family":"Morresi-Hauf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"3061","published-online":{"date-parts":[[2017,11,30]]},"reference":[{"key":"ref-1701481","doi-asserted-by":"crossref","unstructured":"Nicholson AG, Baandrup U, Florio R, et al. Malignant myxoid endobronchial tumour: a report of two cases with a unique histological pattern. Histopathology 1999; 35: 313-318.","DOI":"10.1046\/j.1365-2559.1999.00724.x"},{"key":"ref-1701482","doi-asserted-by":"crossref","unstructured":"Thway K, Nicholson AG, Lawson K, et al. Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion: a new tumor entity. Am J Surg Pathol 2011; 35: 1722-1732.","DOI":"10.1097\/PAS.0b013e318227e4d2"},{"key":"ref-1701483","unstructured":"Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG eds. WHO Classification of Tumours of the Lung, Pleura, Thymus and Heart. 4th ed. IARC Press, Lyon 2015."},{"key":"ref-1701484","doi-asserted-by":"crossref","unstructured":"Inayama Y, Hayashi H, Ogawa N, et al. Low-grade pulmonary myxoid sarcoma of uncertain histogenesis. Pathol Int 2001; 51: 204-210.","DOI":"10.1046\/j.1440-1827.2001.01180.x"},{"key":"ref-1701485","doi-asserted-by":"crossref","unstructured":"Zhou Q, Lu G, Liu A, et al. Extraskeletal myxoid chondrosarcoma in the lung: asymptomatic lung mass with severe anemia. Diagn Pathol 2012; 7: 112.","DOI":"10.1186\/1746-1596-7-112"},{"key":"ref-1701486","doi-asserted-by":"crossref","unstructured":"Matsukuma S, Hisaoka M, Obara K, et al. Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion, resembling extraskeletal myxoid chondrosarcoma: Case report with a review of literature. Pathol Int 2012; 62: 817-822.","DOI":"10.1111\/pin.12014"},{"key":"ref-1701487","doi-asserted-by":"crossref","unstructured":"Smith SC, Palanisamy N, Betz BL, et al. At the intersection of primary pulmonary myxoid sarcoma and pulmonary angiomatoid fibrous histiocytoma: observations from three new cases. Histopathology 2014; 65: 144-146.","DOI":"10.1111\/his.12354"},{"key":"ref-1701488","doi-asserted-by":"crossref","unstructured":"Jeon YK, Moon KC, Park SH, et al. Primary pulmonary myxoid sarcomas with EWSR1-CREB1 translocation might originate from primitive peribronchial mesenchymal cells undergoing (myo)fibroblastic differentiation. Virchows Arch 2014; 465: 453-461.","DOI":"10.1007\/s00428-014-1645-z"},{"key":"ref-1701489","unstructured":"Lucas DR, Stenman G. Extraskeletal Myxoid Chondrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn CW and Mertens F (eds.). WHO Classification of tumors of soft tissue and bone. (Herndon, VA: Stylus Publishing) 2013; 223-224."},{"key":"ref-1701490","doi-asserted-by":"crossref","unstructured":"Thway K, Nicholson AG, Wallace WA, et al. Endobronchial pulmonary angiomatoid fibrous histiocytoma: two cases with EWSR1-CREB1 and EWSR1-ATF1 fusions. Am J Surg Pathol 2012; 36: 883-888.","DOI":"10.1097\/PAS.0b013e31825485c5"},{"key":"ref-1701491","doi-asserted-by":"crossref","unstructured":"Schaefer IM, Fletcher CD. Myxoid variant of so-called angiomatoid \u201cmalignant fibrous histiocytoma\u201d: clinicopathologic characterization in a series of 21 cases. Am J Surg Pathol 2014; 38: 816-823.","DOI":"10.1097\/PAS.0000000000000172"},{"key":"ref-1701492","doi-asserted-by":"crossref","unstructured":"Jo VY, Fletcher CD. Myoepithelial neoplasms of soft tissue: an updated review of the clinicopathologic, immunophenotypic, and genetic features. Head Neck Pathol 2015; 9: 32-38.","DOI":"10.1007\/s12105-015-0618-0"},{"key":"ref-1701493","doi-asserted-by":"crossref","unstructured":"Falk N, Weissferdt A, Kalhor N, et al. Primary Pulmonary Salivary Gland-type Tumors: A Review and Update. Adv Anat Pathol. 2016; 23: 13-23.","DOI":"10.1097\/PAP.0000000000000099"},{"key":"ref-1701494","doi-asserted-by":"crossref","unstructured":"Kalhor N, Suster S, Moran CA. Primary pulmonary chondrosarcomas: a clinicopathologic study of 4 cases. Hum Pathol 2011; 42: 1629-1634.","DOI":"10.1016\/j.humpath.2011.01.009"},{"key":"ref-1701495","doi-asserted-by":"crossref","unstructured":"Herpel E, Rieker RJ, Dienemann H, et al. SMARCA4 and SMARCA2 deficiency in non-small cell lung cancer: immunohistochemical survey of 316 consecutive specimens. Ann Diagn Pathol 26; 2017: 47-51.","DOI":"10.1016\/j.anndiagpath.2016.10.006"},{"key":"ref-1701496","doi-asserted-by":"crossref","unstructured":"Agaimy A. The expanding family of SMARCB1(INI1)-deficient neoplasia: implications of phenotypic, biological, and molecular heterogeneity. Adv Anat Pathol 2014; 21: 394-410.","DOI":"10.1097\/PAP.0000000000000038"}],"container-title":["Polish Journal of Pathology"],"original-title":[],"link":[{"URL":"https:\/\/www.polishjournalofpathology.com\/pdf-76574-136935","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.polishjournalofpathology.com\/pdf-76574-136935","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T10:09:35Z","timestamp":1782209375000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.polishjournalofpathology.com\/EWSR1-fusion-negative-SMARCB1-deficient-primary-pulmonary-myxoid-sarcoma,76574,0,2.html"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,11,30]]},"references-count":16,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2017]]}},"alternative-id":["76574"],"URL":"https:\/\/doi.org\/10.5114\/pjp.2017.71535","relation":{},"ISSN":["1233-9687","2084-9869"],"issn-type":[{"value":"1233-9687","type":"print"},{"value":"2084-9869","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,11,30]]}}}