{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T12:29:37Z","timestamp":1786969777159,"version":"build-2736575974"},"reference-count":9,"publisher":"BMJ","issue":"8","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,8,2]]},"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:p>Primary pulmonary synovial sarcoma (PPSS) is a rare and aggressive malignancy accounting for 0.5% of lung cancers, with a 5-year survival rate of only 30%-50%. Diagnosis is challenging as it has no specific clinical or radiological features that distinguish it from other lung malignancies.<\/jats:p>\n                  <jats:p>We report a man in his 20s who presented with a 6-week history of cough and haemoptysis. Initial chest CT revealed a 2.2 cm endobronchial lesion in the left mainstem bronchus. Bronchoscopy confirmed the obstructing mass but initial biopsy was non-diagnostic. Positron emission tomography-CT scan demonstrated rapid tumour enlargement to 8.2 cm without evidence of distant hypermetabolic activity. CT-guided biopsy revealed spindle cell neoplasm, and immunohistochemistry along with SS18 fluorescence-in-situ-hybridisation confirmed a low-grade PPSS following a multidisciplinary team evaluation. Despite neoadjuvant chemotherapy, the tumour progressed; hence, he underwent sleeve pneumonectomy. This case highlights the importance of early referral to specialised lung centres and multidisciplinary care to enable timely diagnosis and improve outcomes in patients with rare pulmonary malignancies.<\/jats:p>","DOI":"10.1136\/bcr-2026-273841","type":"journal-article","created":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T12:20:19Z","timestamp":1786969219000},"page":"e273841","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["When rare becomes real: diagnostic challenges and use of a multidisciplinary team-based decision in primary pulmonary synovial sarcoma"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-8212-0543","authenticated-orcid":false,"given":"Bryan Leonard Maniago","family":"Quizon","sequence":"first","affiliation":[{"name":"Department of Medicine, University of the Philippines Manila, Manila, Philippines"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noreen Ada","family":"Mangugan","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of the Philippines Manila, Manila, Philippines"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gene Philip Louie Calderon","family":"Ambrocio","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of the Philippines Manila, Manila, Philippines"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lenora C","family":"Fernandez","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of the Philippines Manila, Manila, Philippines"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,8,17]]},"reference":[{"key":"2026081705201125000_19.8.e273841.1","article-title":"A Pulmonary Zebra: Adult Primary Pulmonary Synovial Sarcoma","volume":"2022","author":"Sandra","year":"2022","journal-title":"Case Rep Oncol Med"},{"key":"2026081705201125000_19.8.e273841.2","doi-asserted-by":"crossref","first-page":"63","DOI":"10.4103\/2229-516X.174019","article-title":"Primary pulmonary synovial sarcoma: A case report and review of literature","volume":"6","author":"Bhattacharya","year":"2016","journal-title":"Int J Appl Basic Med Res"},{"key":"2026081705201125000_19.8.e273841.3","doi-asserted-by":"crossref","first-page":"64","DOI":"10.4103\/IJPM.IJPM_851_18","article-title":"Comparison between Fluorescence in-situ Hybridization (FISH), Reverse Transcriptase PCR (RT-PCR) and fragment analysis, for detection of t (X; 18) (p11; q11) translocation in synovial sarcomas","volume":"63","author":"Shetty","year":"2020","journal-title":"Indian J Pathol Microbiol"},{"key":"2026081705201125000_19.8.e273841.4","doi-asserted-by":"crossref","DOI":"10.1111\/crj.70129","article-title":"Combination of Photodynamic and Bronchoscopic Intervention With Radiation Therapy in Treating Pulmonary Pleomorphic Sarcoma: A Case Report","volume":"19","author":"An","year":"2025","journal-title":"Clin Respir J"},{"key":"2026081705201125000_19.8.e273841.5","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1186\/s12931-021-01676-1","article-title":"Educational aspects of rare and orphan lung diseases","volume":"22","author":"Alfaro","year":"2021","journal-title":"Respir Res"},{"key":"2026081705201125000_19.8.e273841.6","first-page":"755","article-title":"Maladies orphelines pulmonaires de la curiosit\u00e9 \u00e0 la sollicitude [Orphan lung diseases: from curiosity to concern]","volume":"188","author":"Cordier","year":"2004","journal-title":"Bull Acad Natl Med"},{"key":"2026081705201125000_19.8.e273841.7","doi-asserted-by":"crossref","first-page":"252","DOI":"10.5582\/irdr.2019.01099","article-title":"Medical students\u2019 knowledge and opinions about rare diseases: A case study from Poland","volume":"8","author":"Domaradzki","year":"2019","journal-title":"Intractable Rare Dis Res"},{"key":"2026081705201125000_19.8.e273841.8","first-page":"162","article-title":"Primary pulmonary sarcoma - long-term treatment outcomes and prognostic factors","volume":"15","author":"Go\u0142ota","year":"2018","journal-title":"Kardiochir Torakochirurgia Pol"},{"key":"2026081705201125000_19.8.e273841.9","doi-asserted-by":"crossref","first-page":"481","DOI":"10.4103\/0973-1482.204883","article-title":"Primary pulmonary synovial sarcoma: A reappraisal","volume":"14","author":"Panigrahi","year":"2018","journal-title":"J Cancer Res Ther"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2026-273841","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T12:20:20Z","timestamp":1786969220000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2026-273841"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8]]},"references-count":9,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2026,8,17]]},"published-print":{"date-parts":[[2026,8]]}},"alternative-id":["10.1136\/bcr-2026-273841"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2026-273841","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8]]}}}