{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T04:48:02Z","timestamp":1760244482579,"version":"build-2065373602"},"reference-count":14,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2022,11,17]],"date-time":"2022-11-17T00:00:00Z","timestamp":1668643200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diseases"],"abstract":"<jats:p>Primary mediastinal sarcoma is a rare tumour that usually presents with nonspecific symptoms such as hoarseness, dyspnoea, and chest pain. Superior vena cava (SVC) syndrome is an extremely uncommon complication that is caused by the compression, invasion, and thrombosis of the SVC or brachiocephalic veins. SVC syndrome can present as asymptomatic cases or as rare life-threatening emergencies with upper airway obstruction and increased intracranial pressure. This report describes the case of a 58-year-old female who presented with swelling of the face, neck, and upper limbs associated with dyspnoea on exertion. The radiological investigations revealed a large well-defined central necrotic peripherally enhancing lesion in the superior mediastinum extending anteriorly with the compression of brachiocephalic veins. A histopathological examination detected spindle cells arranged in fascicles with nuclear atypia with immunohistochemistry positive for creatine kinase (CK), smooth muscle actin (SMA), desmin and CD99. These findings established the diagnosis of a mediastinal monophasic synovial sarcoma with SVC obstruction. The patient was initiated on palliative radiotherapy for the management of the SVC, followed by systemic biological treatment with the tyrosine kinase inhibitor pazopanib, and was clinically improved. It is essential to promptly diagnose and treat this condition, especially when SVC syndrome manifests.<\/jats:p>","DOI":"10.3390\/diseases10040105","type":"journal-article","created":{"date-parts":[[2022,11,18]],"date-time":"2022-11-18T03:29:50Z","timestamp":1668742190000},"page":"105","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Primary Mediastinal Monophasic Spindle-Cell Synovial Sarcoma with Superior Venacaval Obstruction"],"prefix":"10.3390","volume":"10","author":[{"given":"Deepak","family":"Madi","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, 203, Light House Hill Road, Hampankatta, Mangaluru 575001, Karnataka, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6627-1987","authenticated-orcid":false,"given":"Nikhil Victor","family":"Dsouza","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, 203, Light House Hill Road, Hampankatta, Mangaluru 575001, Karnataka, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7386-0384","authenticated-orcid":false,"given":"Matthew Antony","family":"Manoj","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, 203, Light House Hill Road, Hampankatta, Mangaluru 575001, Karnataka, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Basavaprabhu","family":"Achappa","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, 203, Light House Hill Road, Hampankatta, Mangaluru 575001, Karnataka, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2512-6131","authenticated-orcid":false,"given":"Stergios","family":"Boussios","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Medway NHS Foundation Trust, Windmill Road, Gillingham ME7 5NY, UK"},{"name":"School of Cancer & Pharmaceutical Sciences, Faculty of Life Sciences & Medicine, King\u2019s College London, London SE1 9RT, UK"},{"name":"AELIA Organization, 9th Km Thessaloniki-Thermi, 57001 Thessaloniki, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1016\/j.cdip.2006.07.001","article-title":"Pulmonary and thoracic sarcomas","volume":"12","author":"Salter","year":"2006","journal-title":"Curr. Diagn. Pathol."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Salah, S., Al-Ibraheem, A., Daboor, A., and Al-Hussaini, M. (2013). Synovial sarcoma presenting with huge mediastinal mass: A case report and review of literature. BMC Res. Notes, 6.","DOI":"10.1186\/1756-0500-6-240"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1097\/PAS.0000000000001050","article-title":"Mediastinal Synovial Sarcoma: Clinicopathologic Analysis of 21 Cases With Molecular Confirmation","volume":"42","author":"Terra","year":"2018","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"384","DOI":"10.4103\/0377-4929.81650","article-title":"Poorly differentiated monophasic synovial sarcoma of the mediastinum","volume":"54","author":"Arafah","year":"2011","journal-title":"Indian J. Pathol. Microbiol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1097\/01.pas.0000157934.50936.3e","article-title":"Primary synovial sarcomas of the mediastinum: A clinicopathologic, immunohistochemical, and ultrastructural study of 15 cases","volume":"29","author":"Suster","year":"2005","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_6","first-page":"651813","article-title":"Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature","volume":"2015","author":"Madabhavi","year":"2015","journal-title":"Case Rep. Oncol. Med."},{"key":"ref_7","first-page":"412527","article-title":"Primary synovial sarcomas of the mediastinum: A systematic review and pooled analysis of the published literature","volume":"2014","author":"Salah","year":"2014","journal-title":"ISRN Oncol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1038\/modpathol.3800795","article-title":"Primary pulmonary and mediastinal synovial sarcoma: A clinicopathologic study of 60 cases and comparison with five prior series","volume":"20","author":"Hartel","year":"2007","journal-title":"Mod. Pathol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1016\/j.avsg.2016.12.002","article-title":"Soft Tissue Neoplasms Causing Apparent Venous Thoracic Outlet Syndrome","volume":"42","author":"Mirza","year":"2017","journal-title":"Ann. Vasc. Surg."},{"key":"ref_10","first-page":"94","article-title":"The surprising outcome of a giant primary mediastinal synovial sarcoma treated with neoadjuvant chemotherapy","volume":"5","author":"Balieiro","year":"2013","journal-title":"J. Thorac. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.18203\/2320-6012.ijrms20171298","article-title":"Superior vena cava obstruction as manifestation of synovial cell sarcoma: A case report and review of literature","volume":"5","author":"Dhiman","year":"2017","journal-title":"Int. J. Res. Med. Sci."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1016\/S0140-6736(12)60651-5","article-title":"Pazopanib for metastatic soft-tissue sarcoma (PALETTE): A randomised, double-blind, placebo-controlled phase 3 trial","volume":"379","author":"Blay","year":"2012","journal-title":"Lancet"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"602A","DOI":"10.1378\/chest.1704021","article-title":"Mediastinal Synovial Sarcoma Presenting as SVC Syndrome and Airway Obstruction","volume":"144","author":"Lawson","year":"2013","journal-title":"Chest"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e00811","DOI":"10.1002\/rcr2.811","article-title":"Primary synovial sarcoma of the mediastinum: A poor prognosis in a 14-year-old girl","volume":"9","author":"Saad","year":"2021","journal-title":"Respirol. Case Rep."}],"container-title":["Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2079-9721\/10\/4\/105\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:20:19Z","timestamp":1760145619000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2079-9721\/10\/4\/105"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,17]]},"references-count":14,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["diseases10040105"],"URL":"https:\/\/doi.org\/10.3390\/diseases10040105","relation":{},"ISSN":["2079-9721"],"issn-type":[{"type":"electronic","value":"2079-9721"}],"subject":[],"published":{"date-parts":[[2022,11,17]]}}}