{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T08:28:59Z","timestamp":1778660939598,"version":"3.51.4"},"reference-count":19,"publisher":"BMJ","issue":"4","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2021,3,31]]},"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:p>Angiosarcoma (AS) is a rare malignant tumour representing 1%\u20132% of all sarcomas. Primary AS of superior vena cava (SVC) was reported in two cases worldwide. We report a 69-year-old woman with neck discomfort, headache and dyspnoea for 3\u2009months. CT angiography showed thrombosis in SVC and brachiocephalic veins resulting in an SVC syndrome. The patient began anticoagulant therapy and underwent balloon angioplasty with clinical improvement. Additionally, a positron emission tomography scan confirmed the presence of a mediastinal mass involving the SVC locally. The tumour was excised and a prosthesis was placed on the SVC. Histology revealed a heterogeneous tumour matrix, either myxoid and composed by fusiform cells with vimentin, homogeneous CD31 and a 30% Ki67 immunoexpression, supporting the diagnosis of an AS. Due to multiple complications, the patient never started chemotherapy, and after tumour recurrence, she died within 5\u2009months after diagnosis.<\/jats:p>","DOI":"10.1136\/bcr-2020-241266","type":"journal-article","created":{"date-parts":[[2021,4,16]],"date-time":"2021-04-16T13:09:21Z","timestamp":1618578561000},"page":"e241266","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Primary angiosarcoma of superior vena cava: an unexpected diagnosis after an oncological emergency"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8243-6750","authenticated-orcid":false,"given":"F\u00e1bio","family":"R\u00eago Salgueiro","sequence":"first","affiliation":[{"name":"Medical Oncology, Instituto Portugu\u00eas de Oncologia de Coimbra Francisco Gentil EPE, Coimbra, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"In\u00eas","family":"Vieira","sequence":"additional","affiliation":[{"name":"Endocrinology and Metabolism, Centro Hospitalar Universit\u00e1rio de Coimbra EPE, Coimbra, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"In\u00eas","family":"Gomes","sequence":"additional","affiliation":[{"name":"Medical Oncology, Instituto Portugu\u00eas de Oncologia de Coimbra Francisco Gentil EPE, Coimbra, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maja","family":"Petrova","sequence":"additional","affiliation":[{"name":"Internal Medicine, Centro Hospitalar Universit\u00e1rio de Coimbra EPE, Coimbra, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,4,16]]},"reference":[{"key":"2025082722153144000_14.4.e241266.1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19960601)77:11<2400::AID-CNCR32>3.0.CO;2-Z"},{"key":"2025082722153144000_14.4.e241266.2","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1200\/JCO.2017.74.9812","article-title":"Pathologic angiogenesis of malignant vascular sarcomas: implications for treatment","volume":"36","author":"Khan","year":"2018","journal-title":"J Clin Oncol"},{"key":"2025082722153144000_14.4.e241266.3","doi-asserted-by":"publisher","DOI":"10.1097\/JTO.0b013e3181804791"},{"key":"2025082722153144000_14.4.e241266.4","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1055\/s-0037-1608863","article-title":"Malignant venous obstruction: superior vena cava syndrome and beyond","volume":"34","author":"Friedman","year":"2017","journal-title":"Semin Intervent Radiol"},{"key":"2025082722153144000_14.4.e241266.5","doi-asserted-by":"publisher","DOI":"10.1191\/1358863x03vm507xx"},{"key":"2025082722153144000_14.4.e241266.6","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19830815)52:4<740::AID-CNCR2820520429>3.0.CO;2-P"},{"key":"2025082722153144000_14.4.e241266.7","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(10)70023-1"},{"key":"2025082722153144000_14.4.e241266.8","doi-asserted-by":"crossref","DOI":"10.1259\/bjr.20170039","article-title":"Angiosarcoma: clinical and imaging features from head to toe","volume":"90","author":"Gaballah","year":"2017","journal-title":"Br J Radiol"},{"key":"2025082722153144000_14.4.e241266.9","doi-asserted-by":"crossref","first-page":"1507","DOI":"10.12659\/AJCR.911659","article-title":"A case of undifferentiated sarcoma in the superior vena cava and bilateral cervical veins","volume":"19","author":"Kobayashi","year":"2018","journal-title":"Am J Case Rep"},{"key":"2025082722153144000_14.4.e241266.10","first-page":"1","article-title":"Angiosarcoma of the right atrium with extension to SVC and IVC presenting with complete heart block and significant pericardial effusion","volume":"2016","author":"Vakili","year":"2016","journal-title":"Case Rep Cardiol"},{"key":"2025082722153144000_14.4.e241266.11","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.hjc.2016.11.028","article-title":"Large right atrial angiosarcoma with superior vena cava obstruction and cardiovascular collapse","volume":"57","author":"Jiang","year":"2016","journal-title":"Hellenic J Cardiol"},{"key":"2025082722153144000_14.4.e241266.12","doi-asserted-by":"crossref","first-page":"1862","DOI":"10.1056\/NEJMcp067190","article-title":"Superior vena cava syndrome with malignant causes","volume":"356","author":"Wilson","year":"2007","journal-title":"N Engl J Med Overseas Ed"},{"key":"2025082722153144000_14.4.e241266.13","doi-asserted-by":"crossref","unstructured":"Straka C , Ying J , Kong F-M , et al . 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