{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T07:53:19Z","timestamp":1774511599032,"version":"3.50.1"},"reference-count":13,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:00:00Z","timestamp":1772755200000},"content-version":"vor","delay-in-days":5,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Case Reports"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Primary pericardial angiosarcoma is an exceedingly rare and highly aggressive malignancy arising from pericardial endothelial cells, with a reported median survival of approximately 7 months. Owing to its non\u2010specific clinical presentation, diagnosis is frequently\u00a0delayed, particularly in tuberculosis\u2010endemic regions, where symptoms may be attributed to tuberculous pericarditis. We report the case of a 25\u2010year\u2010old Nepalese male who presented with a six\u2010month history of progressively worsening dyspnea (MMRC grade 1 to 4) and an ECOG performance status of 3. Initial evaluation revealed a large pericardial effusion and a right atrial mass measuring 4.5\u2009\u00d7\u20093.7\u2009\u00d7\u20093.2 cm on MDCT. Based on trace\n                    <jats:italic>Mycobacterium tuberculosis<\/jats:italic>\n                    detection on sputum GeneXpert with indeterminate rifampicin resistance, the patient was initially diagnosed and treated as tuberculous pericarditis. However, symptoms persisted despite 2\u2009weeks of antitubercular therapy. Subsequent pericardiocentesis yielded hemorrhagic fluid; repeat sputum and pericardial fluid testing were negative for tuberculosis, and pericardial fluid cytology was inconclusive for malignancy. Due to persistent diagnostic uncertainty, the patient underwent median sternotomy with creation of a bilateral pleuropericardial window. Histopathological examination revealed high\u2010grade angiosarcoma, with immunohistochemistry positive for CD31, CD34, and ERG, and a Ki\u201067 proliferation index of 30%. Baseline systemic staging showed no definite evidence of distant metastasis. The patient received first\u2010line chemotherapy with ifosfamide and epirubicin. After 3\u2009cycles, remarkable clinical improvement was observed, including improvement in ECOG performance status from 3 to 1, complete resolution of the pericardial effusion, and a 65% reduction in tumor volume. However, following completion of 6\u2009cycles, MDCT demonstrated disease progression with new left para\u2010aortic deposits measuring 5.6\u2009\u00d7\u20092.6 cm. Second\u2010line chemotherapy with nab\u2010paclitaxel and gemcitabine was subsequently initiated, with planned response assessment after 3\u2009cycles. This case highlights the significant diagnostic challenges of pericardial angiosarcoma in tuberculosis\u2010endemic regions and underscores the need to consider malignant etiologies in patients with hemorrhagic pericardial effusion and cardiac masses. Although substantial initial responses to chemotherapy may occur, the disease remains highly aggressive with a high risk of progression. Early histopathological diagnosis and timely initiation of adaptive, multimodal treatment strategies are critical to optimizing clinical outcomes.\n                  <\/jats:p>","DOI":"10.1002\/ccr3.72131","type":"journal-article","created":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T00:01:19Z","timestamp":1773014479000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Primary Pericardial Angiosarcoma Demonstrating Initial Response Followed by Progression on First\u2010Line Chemotherapy: A Case Report"],"prefix":"10.1002","volume":"14","author":[{"given":"Ujjwal Kumar","family":"Thakur","sequence":"first","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-9147-2264","authenticated-orcid":false,"given":"Akash","family":"Subedi","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rheecha","family":"Joshi","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery Shahid Gangalal National Heart Centre  Kathmandu Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sunita","family":"Dhanchha","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sumit","family":"Yadav","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Binaya","family":"Pangyani","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ajaya","family":"Acharya","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, School of Science Kathmandu University  Dhulikhel, Kavre Nepal"},{"name":"Department of Research and Academics Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Punam","family":"KC","sequence":"additional","affiliation":[{"name":"Department of Research and Academics Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9174-7120","authenticated-orcid":false,"given":"Sunil","family":"Shrestha","sequence":"additional","affiliation":[{"name":"Department of Research and Academics Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerim","family":"Prasai","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abish","family":"Adhikari","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anjani Kumar","family":"Jha","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Subhas","family":"Pandit","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simit","family":"Sapkota","sequence":"additional","affiliation":[{"name":"Department of Clinical Oncology Kathmandu Cancer Center  Tathali Bhaktapur Nepal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,3,6]]},"reference":[{"issue":"7","key":"e_1_2_14_2_1","article-title":"Primary Cardiac Angiosarcoma: A Review","volume":"15","author":"Kumari N.","year":"2023","journal-title":"Cureus"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1054-8807(03)00087-5"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ssu.2980100511"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/JTO.0b013e3181e59a91"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.3332\/ecancer.2020.1056"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.3389\/fcvm.2024.1344975"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11886-020-01420-z"},{"key":"e_1_2_14_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40001-019-0389-2"},{"key":"e_1_2_14_10_1","volume-title":"WHO Consolidated Operational Handbook on Tuberculosis. 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