{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T06:05:20Z","timestamp":1780380320288,"version":"3.54.1"},"reference-count":25,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T00:00:00Z","timestamp":1780358400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm lacking a standard systemic therapy. Pulmonary EHE (PEH) may be aggressive if accompanied by serosal effusion and has occasionally been reported in association with hypertrophic pulmonary osteoarthropathy (HPOA). Here, we report a case of a 33-year-old woman who had never smoked and presented with chronic cough, arthralgia, and digital clubbing. Imaging revealed a left upper lobe mass with mediastinal invasion, lymphadenopathy, and pericardial effusion. The patient developed cardiac tamponade that required emergent pericardial drainage. Histopathology from the transbronchial biopsy and mediastinal lymph node sampling showed an epithelioid endothelial tumor positive for CD31, D2-40, factor VIII, and ERG with nuclear CAMTA1 expression, confirming EHE. Bone scintigraphy and ankle magnetic resonance imaging revealed symmetric periosteal-predominant abnormalities consistent with HPOA. Zoledronic acid was administered for HPOA, and paclitaxel\/carboplatin plus bevacizumab was initiated with palliative intent. Follow-up imaging during treatment showed marked reduction of the pericardial and pleural effusions, with improvement in arthralgia and activities of daily living. Although the benefit was transient, this case suggests that systemic chemotherapy in combination with bevacizumab may have contributed not only to temporary effusion control in advanced PEH but also to relief of PEH-associated HPOA, while concomitant zoledronic acid may also have supported symptom improvement.<\/jats:p>","DOI":"10.3389\/fonc.2026.1855346","type":"journal-article","created":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T05:43:48Z","timestamp":1780379028000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical benefit of paclitaxel\/carboplatin plus bevacizumab with zoledronic acid in pulmonary epithelioid hemangioendothelioma complicated by hypertrophic pulmonary osteoarthropathy and cardiac tamponade: a case report"],"prefix":"10.3389","volume":"16","author":[{"given":"Saki","family":"Tsuchimochi","sequence":"first","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiromichi","family":"Nakajima","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]},{"name":"Department of Experimental Therapeutics, National Cancer Center Hospital East","place":["Kashiwa, Japan"]},{"name":"Department of General Internal Medicine, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukako","family":"Hattori","sequence":"additional","affiliation":[{"name":"Department of Palliative Medicine, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shioto","family":"Oda","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Radiology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"Imada","sequence":"additional","affiliation":[{"name":"Department of Pathology and Clinical Laboratories, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tetsuro","family":"Taki","sequence":"additional","affiliation":[{"name":"Department of Pathology and Clinical Laboratories, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Matsuoka","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shogo","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Hirota","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mai","family":"Shimura","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Misao","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chikako","family":"Funasaka","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]},{"name":"Department of Experimental Therapeutics, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenichi","family":"Harano","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]},{"name":"Department of Experimental Therapeutics, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuaki","family":"Matsubara","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoichi","family":"Naito","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]},{"name":"Department of Experimental Therapeutics, National Cancer Center Hospital East","place":["Kashiwa, Japan"]},{"name":"Department of General Internal Medicine, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ako","family":"Hosono","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]},{"name":"Department of Pediatric Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genichiro","family":"Ishii","sequence":"additional","affiliation":[{"name":"Department of Pathology and Clinical Laboratories, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toru","family":"Mukohara","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital East","place":["Kashiwa, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,2]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.esmoop.2021.100170","article-title":"Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts","volume":"6","author":"Stacchiotti","year":"2021","journal-title":"ESMO Open"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2025.116100","article-title":"Incidence, prevalence and treatment of patients with epithelioid haemangioendothelioma (EHE) in Canada: A Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) and Multi-pronged Canadian Research in Epithelioid Haemangioendothelioma (PRO_CARE EHE) study","volume":"231","author":"Weadick","year":"2025","journal-title":"Eur J Cancer"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/j.critrevonc.2025.105021","article-title":"Translating molecular mechanisms of epithelioid hemangioendothelioma to improve patient outcome","volume":"217","author":"Beretta","year":"2026","journal-title":"Crit Rev Oncol Hematol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinsp.2025.100789","article-title":"Pulmonary epithelioid haemangioendothelioma: A comprehensive review of clinical and molecular advances","volume":"80","author":"Tang","year":"2025","journal-title":"Clinics (Sao Paulo)"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1148\/rg.2017160052","article-title":"Hypertrophic osteoarthropathy: Clinical and imaging features","volume":"37","author":"Yap","year":"2017","journal-title":"Radiographics"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1016\/s0929-693x(03)00276-8","article-title":"Hypertrophic pulmonary osteo-arthropathy revealed by a pulmonary epithelioid hemangioendothelioma in a 15-year-old girl","volume":"10","author":"Chartier","year":"2003","journal-title":"Arch Pediatr"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1136\/thx.54.6.560","article-title":"Epithelioid haemangioendothelioma","volume":"54","author":"Ledson","year":"1999","journal-title":"Thorax"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2019.09.033","article-title":"A 49-year-old man with cough and hand, wrist, and knee pain","volume":"157","author":"Abramian","year":"2020","journal-title":"Chest"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.3346\/jkms.2004.19.3.484","article-title":"A case of hypertrophic osteoarthropathy associated with epithelioid hemangioendothelioma","volume":"19","author":"Kim","year":"2004","journal-title":"J Korean Med Sci"},{"key":"B10","article-title":"Hypertrophic osteoarthropathy disclosing hepatic and pulmonary multifocal epithelioid hemangioendothelioma","volume":"60","author":"Zufferey","year":"1993","journal-title":"Rev Rhum Ed Fr"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1843.2009.01512.x","article-title":"Pulmonary epithelioid haemangioendothelioma mimicking central lung cancer","volume":"14","author":"Shang","year":"2009","journal-title":"Respirology"},{"key":"B12","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1007\/s10067-010-1563-7","article-title":"Review of current therapies for secondary hypertrophic pulmonary osteoarthropathy","volume":"30","author":"Nguyen","year":"2011","journal-title":"Clin Rheumatol"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1007\/s12020-024-03804-5","article-title":"Role of bisphosphonates in hypertrophic osteoarthropathy: a systematic review","volume":"85","author":"Palui","year":"2024","journal-title":"Endocrine"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2018.03.045","article-title":"Diagnosing epithelioid hemangioendothelioma with pericardial involvement","volume":"106","author":"Balansay","year":"2018","journal-title":"Ann Thorac Surg"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1136\/bcr.09.2008.0955","article-title":"Cardiac tamponade caused by epithelioid haemangioendothelioma","volume":"2009","author":"Chen","year":"2009","journal-title":"BMJ Case Rep"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.4103\/heartviews.heartviews_96_19","article-title":"Right atrial pseudoaneurysm complicating epithelioid hemangioendothelioma","volume":"21","author":"Moidy","year":"2020","journal-title":"Heart Views"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mds237","article-title":"An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas","volume":"24","author":"Agulnik","year":"2013","journal-title":"Ann Oncol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.21037\/apm-21-2623","article-title":"Clinical efficacy of bevacizumab combined with cisplatin in the treatment of Malignant pleural effusion and ascites caused by lung cancer: a randomized trial","volume":"10","author":"Zhou","year":"2021","journal-title":"Ann Palliat Med"},{"key":"B19","doi-asserted-by":"publisher","first-page":"278","DOI":"10.1186\/s12957-025-03887-y","article-title":"Intracavitary perfusion with bevacizumab plus cisplatin versus cisplatin alone for Malignant pleural effusion in lung cancer patients: a meta-analysis of randomized controlled trials","volume":"23","author":"Han","year":"2025","journal-title":"World J Surg Oncol"},{"key":"B20","article-title":"The role of angiogenesis in Malignant pleural effusion: from basic research to clinical application","volume":"12","author":"Zhao","year":"2022","journal-title":"Am J Cancer Res"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.28109","article-title":"Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group (GSF\/GETO)","volume":"119","author":"Chevreau","year":"2013","journal-title":"Cancer"},{"key":"B22","doi-asserted-by":"publisher","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":"van der Graaf","year":"2012","journal-title":"Lancet"},{"key":"B23","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1093\/annonc\/mdw388.16","article-title":"Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis","volume":"56","author":"Kollar","year":"2017","journal-title":"Acta Oncol"},{"key":"B24","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1007\/s00432-025-06208-8","article-title":"First-line pazopanib in patients with metastatic epithelioid hemangioendothelioma: a retrospective single-center analysis","volume":"151","author":"Burkhard-Meier","year":"2025","journal-title":"J Cancer Res Clin Oncol"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1002\/cam4.3807","article-title":"Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature","volume":"10","author":"Frezza","year":"2021","journal-title":"Cancer Med"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1855346\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T05:43:49Z","timestamp":1780379029000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1855346\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,2]]},"references-count":25,"alternative-id":["10.3389\/fonc.2026.1855346"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1855346","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,2]]},"article-number":"1855346"}}