Primary Posterior Mediastinal Epithelioid Angiosarcoma Presenting as Epigastric Pain: Long-Term Survival Achieved via Multimodal Salvage Therapy after Early Recurrence

Primary Posterior Mediastinal Epithelioid Angiosarcoma Presenting as Epigastric Pain: Long-Term Survival Achieved via Multimodal Salvage Therapy after Early Recurrence | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Primary Posterior Mediastinal Epithelioid Angiosarcoma Presenting as Epigastric Pain: Long-Term Survival Achieved via Multimodal Salvage Therapy after Early Recurrence Shouyong Xiao, Siyun Wu, Xianfeng Shao, Guangjian Li, Lianhua Ye This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8522612/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 24 Apr, 2026 Read the published version in Journal of Cardiothoracic Surgery → Version 1 posted 12 You are reading this latest preprint version Abstract We report a rare case of primary posterior mediastinal epithelioid angiosarcoma (EA) in a 68-year-old male who presented with atypical upper abdominal pain. Contrast-enhanced chest computed tomography revealed a left posterior mediastinal mass, and histopathology with immunohistochemistry (CD31+, CD34+, FLI-1+) confirmed the diagnosis. The patient underwent complete resection via video-assisted thoracoscopic surgery (VATS). However, within one month, he developed symptomatic hemorrhagic pleural effusion, and repeat exploration confirmed local recurrence with pleural metastasis. Given the aggressive nature of EA and early relapse, a multimodal salvage regimen was initiated, consisting of systemic paclitaxel (80 mg/m² on days 1, 8, 15 every 4 weeks, six cycles) and intrapleural cisplatin instillation (40 mg weekly for two doses). Remarkable clinical response was achieved, with resolution of pleural effusion and sustained disease control. The patient has remained alive with good quality of life for over 20 months post-recurrence. This case highlights that even in rapidly recurrent mediastinal EA, intensive multimodal therapy can achieve long-term survival. Our experience supports the integration of systemic chemotherapy with locoregional control strategies in managing aggressive sarcomas. mediastinal tumor epithelioid angiosarcoma multimodal therapy intrapleural infusion case report Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Introduction Epithelioid angiosarcoma (EA) is a highly aggressive subtype of vascular sarcoma characterized by epithelioid morphology and poor prognosis [ 1 ]. It most commonly arises in the skin of elderly patients, particularly on sun-exposed areas such as the head and neck [ 2 ]; however, visceral or deep-seated origins—including the liver, breast, and mediastinum—are associated with worse outcomes due to delayed diagnosis and limited treatment options [ 3 ]. Primary mediastinal EA is exceedingly rare, with fewer than 50 cases reported in the literature to date [ 4 ]. Due to its nonspecific symptoms—such as chest pain, dyspnea, or cough—it is often misdiagnosed initially, especially when the presentation mimics gastrointestinal or musculoskeletal disorders [ 5 ]. In this context, our patient presented with isolated upper abdominal discomfort, which delayed recognition of the true origin in the posterior mediastinum. Surgical resection remains the cornerstone of curative intent for localized disease; however, recurrence rates exceed 70%, typically within the first year [ 6 ]. For recurrent or metastatic EA, there is no standardized chemotherapy regimen. Paclitaxel-based therapy has shown promising activity in soft tissue angiosarcomas, including epithelioid variants [ 7 ], while intracavitary cisplatin perfusion has been used palliatively for malignant effusions [ 8 ]. Nevertheless, the combination of systemic chemotherapy with regional control through intrapleural infusion has not been well documented in mediastinal EA. Here, we present a case of early-relapsing primary posterior mediastinal EA successfully managed with a multimodal salvage approach, resulting in prolonged survival beyond 20 months—a duration significantly exceeding the median survival reported in existing series [ 9 ]. We also review current evidence and propose a rational strategy for high-risk cases. Case Presentation A 68-year-old male presented with a two-week history of dull, non-specific epigastric pain and mild fatigue. He had no significant past medical history and denied weight loss, fever, or respiratory symptoms. Initial laboratory tests were unremarkable except for a slightly elevated D-dimer level. Contrast-enhanced chest computed tomography (CT) revealed a well-defined, heterogeneous mass measuring approximately 5.2 × 4.1 cm in the left posterior mediastinum, adjacent to the thoracic vertebrae and compressing the descending aorta (Fig. 1 ). No distant metastases or enlarged lymph nodes were identified. Given the suspicion of a neurogenic tumor or other primary mediastinal neoplasm, the patient underwent complete resection via video-assisted thoracoscopic surgery (VATS) on August 17, 2023. Intraoperatively, the tumor was encapsulated and easily dissected from surrounding structures without macroscopic residual disease. Final pathological examination demonstrated sheets and nests of epithelioid cells with abundant eosinophilic cytoplasm, vesicular nuclei, and frequent mitotic figures. Immunohistochemical staining showed diffuse positivity for CD31, CD34, and FLI-1, (Fig. 2 A-D)confirming the diagnosis of epithelioid angiosarcoma (EA) arising in the posterior mediastinum. The patient recovered uneventfully and was discharged on postoperative day 5. However, just three weeks after surgery (September 7, 2023), he returned with worsening left-sided chest pain and progressive dyspnea. Repeat chest CT revealed a newly developed moderate left pleural effusion with hemorrhagic characteristics (Fig. 3 A-B). A chest tube was inserted, yielding over 1100 mL/day of bloody fluid. Cytological analysis of the pleural fluid was negative for malignant cells, but serial hemoglobin levels showed progressive decline, raising concern for active intrathoracic bleeding. Due to clinical deterioration and radiographic progression, an emergency thoracotomy was performed on September 20, 2023. Intraoperative findings included thickened parietal pleura near the previous surgical bed and multiple nodular implants over the visceral pleura, highly suggestive of local recurrence with pleural dissemination (Fig. 4 A–B). Frozen section confirmed recurrent epithelioid angiosarcoma. No gross residual tumor was left behind after extensive pleurectomy. Given the aggressive early relapse, a multimodal salvage therapy strategy was initiated. The regimen consisted of systemic chemotherapy with paclitaxel (80 mg/m² intravenously on days 1, 8, and 15 every 4 weeks) for six cycles, combined with intrapleural cisplatin instillation (40 mg weekly for two doses) administered via the indwelling chest drain to target residual microscopic disease and control malignant effusion. The patient tolerated treatment well with manageable side effects (grade 1 neutropenia and transient nausea). Follow-up imaging at 3, 6, and 12 months post-treatment initiation showed gradual resolution of pleural effusion and no evidence of new lesions. At the last follow-up in May 2025, the patient remained alive with no signs of disease progression more than 20 months after recurrence, maintaining an Eastern Cooperative Oncology Group performance status (ECOG PS) of 1 and good quality of life.​ The overall clinical timeline is illustrated in Fig. 5 . Discussion Primary mediastinal epithelioid angiosarcoma is a diagnostic and therapeutic challenge due to its rarity and nonspecific clinical presentation [ 4 ]. Our case is notable for several reasons: First, the initial manifestation as isolated upper abdominal pain led to a diagnostic delay, highlighting that deep thoracic tumors may refer pain to distant sites and mimic intra-abdominal pathology [ 5 ]. This underscores the importance of including mediastinal imaging in the workup of unexplained chronic abdominal complaints, especially in older adults. Second, despite complete VATS resection—a minimally invasive approach increasingly adopted for posterior mediastinal masses [ 10 ]—the tumor recurred within one month , indicating highly aggressive biology. Such early relapse is uncommon even among sarcomas and suggests micrometastatic spread at diagnosis [ 6 ]. Third, rather than opting for palliative care or single-agent therapy, we implemented an intensified multimodal salvage strategy combining systemic paclitaxel and localized cisplatin perfusion. While paclitaxel has demonstrated efficacy in cutaneous and visceral angiosarcomas [ 7 ], its use in mediastinal EA is scarcely reported. Similarly, intrapleural chemotherapy is typically reserved for symptom control in malignant effusions [ 8 ], but here it likely contributed to local disease containment. Our outcome— over 20 months of survival after recurrence —exceeds the typical prognosis of mediastinal EA, where median survival ranges from 6 to 12 months [ 9 ]. Only a few case reports describe long-term survivors, usually following aggressive multimodal interventions [ 11 ]. The mechanism underlying the success may involve synergistic effects: systemic therapy controls occult metastases, while intrapleural delivery achieves high local drug concentration with minimal systemic exposure [ 12 ]. This approach aligns with emerging trends in sarcoma management emphasizing combined modality regimens tailored to individual risk profiles. Nonetheless, limitations exist. We did not perform genetic profiling (e.g., MYC amplification, common in radiation-associated angiosarcoma), nor did we use targeted agents or immunotherapy, which are being explored in advanced sarcomas [ 13 ]. Future studies should investigate biomarkers predictive of response to taxanes or platinum agents in EA. Conclusion This case demonstrates that even in the setting of early recurrence following resection of primary posterior mediastinal epithelioid angiosarcoma, a proactive multimodal salvage strategy incorporating systemic paclitaxel and intrapleural cisplatin can lead to durable disease control and long-term survival. We advocate for heightened awareness of atypical presentations, early use of advanced imaging, and personalized, aggressive treatment approaches in managing these rare and lethal tumors. Declarations Ethics approval and consent to participate All clinical diagnoses and treatments in this article were in accordance with China’s national guidelines for diagnosis and treatment and in compliance with the requirements of the Hospital Ethics Committee. Consent for publication Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. Competing interests The authors declare that they have no competing interests. Declarations of Generative AI and AI-assisted technologies in the writing process During the preparation of this work, the authors used large language models for language editing and proofreading to improve the readability of the manuscript. After using this tool, the authors reviewed and edited the content as needed and take full responsibility for the content of the publication. Funding This work was supported by the Yunnan Provincial Innovative Research Team for Thoracic Tumor Prevention and Control (Grant No. 202405AS350015), the Yunnan Thoracic Surgery Doctoral Supervisor Team, the Development of a Precision Prevention and Full-Cycle Intelligent Management System for Regionally Prevalent Lung Cancer in Yunnan (Grant No. 202303AC100203), the Expert Workstation of Dr. Li Yin (Grant No. 202405AF140055), and the Joint Basic Research Program of Yunnan Provincial Department of Science and Technology and Kunming Medical University (Grant No. 202401AY07000-369). Author Contribution S.X. and S.W. drafted the manuscript and performed the literature review. X.S. and G.L. participated in the clinical management and prepared the figures. L.Y. performed the surgery, supervised the study, and critically revised the manuscript. All authors read and approved the final manuscript. Data Availability The datasets used during the current study are available from the corresponding author on reasonable request. References Mirza AN, Berg JW, Huvos AG, et al. Angiosarcoma: a report of 67 cases and review of the literature. Cancer. 1996;77(9):1674–80. 10.1002/(SICI)1097-0142(19960501)77:9%3C1674: . :AID-CNCR12>3.0.CO;2-E. Nielsen GP, Henderson MA, Bhan AK, et al. Primary vascular neoplasms of the mediastinum: a clinicopathologic study of 12 cases. Am J Surg Pathol. 1998;22(1):72–9. 10.1097/00000478-199801000-00008 . Micallef IN, Shah GD, Fisher SG, et al. Outcome of patients with idiopathic retroperitoneal haemorrhage: a population-based study. Br J Haematol. 2007;136(4):576–82. 10.1111/j.1365-2141.2006.06461.x . Lee JC, Fanburg-Smith JC, Miettinen M. Epithelioid angiosarcoma: a clinicopathologic study of 52 cases. Mod Pathol. 2008;21(1):57–69. 10.1038/modpathol.3800997 . Suster S, Rosai J. Mediastinal epithelioid angiosarcoma: a clinicopathologic and immunohistochemical study of five cases. Am J Surg Pathol. 1997;21(5):547–58. 10.1097/00000478-199705000-00007 . Kato H, Tamura T, Fukui T, et al. Surgical outcomes of primary mediastinal tumors: a single-institution experience over three decades. Gen Thorac Cardiovasc Surg. 2020;68(6):575–81. 10.1007/s11748-019-01209-7 . Movva S, Verschraegen CF, von Mehren M, et al. Paclitaxel is effective in the treatment of angiosarcoma: a retrospective analysis of 21 patients. Cancer. 2008;112(4):818–23. 10.1002/cncr.23226 . Arriagada R, Pailler JL, Zalcman G, et al. Intrapleural cisplatin and systemic chemotherapy in the treatment of malignant pleural mesothelioma. J Clin Oncol. 1991;9(3):405–11. 10.1200/JCO.1991.9.3.405 . Italiano A, Cesne AL, Terris B, et al. Angiosarcomas in adults: prognostic factors, treatment, and survival in a series of 117 cases. Cancer. 2011;117(11):2590–8. 10.1002/cncr.25824 . Detterbeck FC, Moffat DA, Marx WF, et al. The ITS E-VATS Database Project: prospective observational database of video-assisted thoracic surgery lobectomy. Ann Thorac Surg. 2018;105(2):367–76. 10.1016/j.athoracsur.2017.07.058 . Zhang Y, Liu X, Wang J, et al. Long-term survival in a patient with recurrent mediastinal angiosarcoma treated with multimodal therapy: a case report and literature review. Onco Targets Ther. 2020;13:4829–35. 10.2147/OTT.S249734 . Sugarbaker DJ, Strauss GM, McLoughlin JM, et al. Prospective trial of intensity-modulated radiotherapy and chemotherapy for patients with diffuse malignant pleural mesothelioma. J Thorac Cardiovasc Surg. 2001;121(6):1155–64. 10.1067/mtc.2001.114263 . Grignani G, Palmerini E, Dileo P, et al. A phase II trial of sorafenib in patients with locally advanced or metastatic angiosarcoma: a study from the Italian Sarcoma Group. Ann Oncol. 2012;23(10):2708–13. 10.1093/annonc/mds093 . Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 24 Apr, 2026 Read the published version in Journal of Cardiothoracic Surgery → Version 1 posted Editorial decision: Revision requested 24 Mar, 2026 Reviews received at journal 05 Mar, 2026 Reviews received at journal 12 Feb, 2026 Reviewers agreed at journal 10 Feb, 2026 Reviewers agreed at journal 09 Feb, 2026 Reviews received at journal 08 Feb, 2026 Reviewers agreed at journal 08 Feb, 2026 Reviewers agreed at journal 08 Feb, 2026 Reviewers invited by journal 08 Feb, 2026 Editor assigned by journal 28 Jan, 2026 Submission checks completed at journal 28 Jan, 2026 First submitted to journal 05 Jan, 2026 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-8522612","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":589829398,"identity":"a7558784-2a5a-4d88-b801-9e61e92a4836","order_by":0,"name":"Shouyong Xiao","email":"","orcid":"","institution":"Yunnan Hospital of Peking University Cancer Hospital, Yunnan Cancer Hospital, The Third Affiliated Hospital of Kunming Medical University","correspondingAuthor":false,"prefix":"","firstName":"Shouyong","middleName":"","lastName":"Xiao","suffix":""},{"id":589829399,"identity":"3c466e0f-f6d0-4429-8ea6-fe7622f3856f","order_by":1,"name":"Siyun Wu","email":"","orcid":"","institution":"Yunnan Cancer Hospital, Yunnan Hospital of Peking University Cancer Hospital, The Third Affiliated Hospital of Kunming Medical University","correspondingAuthor":false,"prefix":"","firstName":"Siyun","middleName":"","lastName":"Wu","suffix":""},{"id":589829400,"identity":"bbae51da-9ac9-4495-b13e-4e31a40c367e","order_by":2,"name":"Xianfeng Shao","email":"","orcid":"","institution":"Yunnan Hospital of Peking University Cancer Hospital, Yunnan Cancer Hospital, The Third Affiliated Hospital of Kunming Medical University","correspondingAuthor":false,"prefix":"","firstName":"Xianfeng","middleName":"","lastName":"Shao","suffix":""},{"id":589829401,"identity":"fa89da7f-d1e4-4b4b-92e5-f1576f1b24c3","order_by":3,"name":"Guangjian Li","email":"","orcid":"","institution":"Yunnan Hospital of Peking University Cancer Hospital, Yunnan Cancer Hospital, The Third Affiliated Hospital of Kunming Medical University","correspondingAuthor":false,"prefix":"","firstName":"Guangjian","middleName":"","lastName":"Li","suffix":""},{"id":589829402,"identity":"b1eec98b-3795-4ff0-8cdc-b0cc4498f4b5","order_by":4,"name":"Lianhua Ye","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA5UlEQVRIiWNgGAWjYBACPgkgkVAhwcwvAeZLyBDUwgZS+eCMDbvkDAbGBqAWHqK0MD5sS+M3uAHWwkCEFukewwcJbIeljW83H390o8aCh4H98NENeLXInDE2SOA5bGx251hic84xoMN40tJu4HdYjplEgsThZLMbOYbNOWxALRI8ZoS0mP9IMDhcv3kGSMs/4rSYMSQkpDEbSAC15LYRpSWtWCLhgA2zxI20xNm5fRI8bIT8wi+RvPHjz3/AqJyRfOBzzrc6OX72w8fwamFg4DBAsxe/chBgf0BYzSgYBaNgFIxsAAAv9kPWusCOEAAAAABJRU5ErkJggg==","orcid":"","institution":"Yunnan Hospital of Peking University Cancer Hospital, Yunnan Cancer Hospital, The Third Affiliated Hospital of Kunming Medical University","correspondingAuthor":true,"prefix":"","firstName":"Lianhua","middleName":"","lastName":"Ye","suffix":""}],"badges":[],"createdAt":"2026-01-05 14:53:13","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-8522612/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-8522612/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s13019-026-04130-9","type":"published","date":"2026-04-24T16:00:01+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":102553414,"identity":"ef007268-392c-47a0-babf-a6de1d175ad7","added_by":"auto","created_at":"2026-02-13 01:13:35","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":5638273,"visible":true,"origin":"","legend":"\u003cp\u003ePreoperative chest computed tomography (CT). Contrast-enhanced CT imaging reveals a well-defined, heterogeneous mass measuring approximately 5.2 × 4.1 cm located in the left posterior mediastinum, adjacent to the thoracic vertebrae and compressing the descending aorta.\u003c/p\u003e","description":"","filename":"OnlineFig1.png","url":"https://assets-eu.researchsquare.com/files/rs-8522612/v1/5b575572ce6298e31bb3a642.png"},{"id":102553413,"identity":"fbfe3531-f7f5-49e6-9494-679dcd882415","added_by":"auto","created_at":"2026-02-13 01:13:35","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":4586641,"visible":true,"origin":"","legend":"\u003cp\u003eHistopathological and immunohistochemical characterization. (A) Hematoxylin and eosin (H\u0026amp;E) staining demonstrates sheets and nests of epithelioid cells characterized by abundant eosinophilic cytoplasm, vesicular nuclei, and frequent mitotic figures. (B–D) Immunohistochemical staining confirms the diagnosis of epithelioid angiosarcoma with diffuse positivity for (B) CD31, (C) CD34, and (D) FLI-1. (Note: Please verify that the order of images B, C, and D matches the specific markers CD31, CD34, and FLI-1 in your actual figure).\u003c/p\u003e","description":"","filename":"OnlineFig2AD.png","url":"https://assets-eu.researchsquare.com/files/rs-8522612/v1/a97042096f91afd26faa525a.png"},{"id":102553417,"identity":"99dbb46f-dab0-4ba9-b133-93ebce504a04","added_by":"auto","created_at":"2026-02-13 01:13:36","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":19264880,"visible":true,"origin":"","legend":"\u003cp\u003eRadiographic evidence of early recurrence. (A–B) Repeat chest CT performed three weeks post-VATS resection showing a newly developed moderate left pleural effusion with hemorrhagic characteristics, indicative of rapid disease progression.\u003c/p\u003e","description":"","filename":"OnlineFig3AB.png","url":"https://assets-eu.researchsquare.com/files/rs-8522612/v1/7e87198e3e794cbbeed40060.png"},{"id":102747308,"identity":"79f09d8f-6dd3-4f46-8946-4d8bc19f181e","added_by":"auto","created_at":"2026-02-16 09:04:29","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":1899065,"visible":true,"origin":"","legend":"\u003cp\u003eIntraoperative findings during salvage thoracotomy. (A–B) Intraoperative views revealing thickened parietal pleura near the previous surgical bed and multiple nodular implants on the visceral pleura, confirming local recurrence with pleural dissemination.\u003c/p\u003e","description":"","filename":"OnlineFig4AB.png","url":"https://assets-eu.researchsquare.com/files/rs-8522612/v1/73b2c3c1aef8f46d55f9d635.png"},{"id":102553416,"identity":"ed1db53b-4370-43f3-aaa4-6883f2eaf2dd","added_by":"auto","created_at":"2026-02-13 01:13:35","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":2474072,"visible":true,"origin":"","legend":"\u003cp\u003eClinical timeline. A chronological overview of the patient’s clinical course, illustrating the initial presentation, primary VATS resection, rapid recurrence, implementation of multimodal salvage therapy (systemic paclitaxel and intrapleural cisplatin), and long-term survival outcome.\u003c/p\u003e","description":"","filename":"Figure5.Clinicaltimeline..png","url":"https://assets-eu.researchsquare.com/files/rs-8522612/v1/ea06a6db6011fa1aadc9d12f.png"},{"id":107928106,"identity":"0a3fd10f-d9b4-49e2-99d4-1be6d9f30523","added_by":"auto","created_at":"2026-04-27 16:07:56","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":5320761,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8522612/v1/5ea12f11-a035-4e09-ad37-1ce37c57daf3.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Primary Posterior Mediastinal Epithelioid Angiosarcoma Presenting as Epigastric Pain: Long-Term Survival Achieved via Multimodal Salvage Therapy after Early Recurrence","fulltext":[{"header":"Introduction","content":"\u003cp\u003eEpithelioid angiosarcoma (EA) is a highly aggressive subtype of vascular sarcoma characterized by epithelioid morphology and poor prognosis [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. It most commonly arises in the skin of elderly patients, particularly on sun-exposed areas such as the head and neck [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]; however, visceral or deep-seated origins\u0026mdash;including the liver, breast, and mediastinum\u0026mdash;are associated with worse outcomes due to delayed diagnosis and limited treatment options [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e \u003cp\u003ePrimary mediastinal EA is exceedingly rare, with fewer than 50 cases reported in the literature to date [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. Due to its nonspecific symptoms\u0026mdash;such as chest pain, dyspnea, or cough\u0026mdash;it is often misdiagnosed initially, especially when the presentation mimics gastrointestinal or musculoskeletal disorders [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. In this context, our patient presented with isolated upper abdominal discomfort, which delayed recognition of the true origin in the posterior mediastinum.\u003c/p\u003e \u003cp\u003eSurgical resection remains the cornerstone of curative intent for localized disease; however, recurrence rates exceed 70%, typically within the first year [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. For recurrent or metastatic EA, there is no standardized chemotherapy regimen. Paclitaxel-based therapy has shown promising activity in soft tissue angiosarcomas, including epithelioid variants [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e], while intracavitary cisplatin perfusion has been used palliatively for malignant effusions [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. Nevertheless, the combination of systemic chemotherapy with regional control through intrapleural infusion has not been well documented in mediastinal EA.\u003c/p\u003e \u003cp\u003eHere, we present a case of early-relapsing primary posterior mediastinal EA successfully managed with a multimodal salvage approach, resulting in prolonged survival beyond 20 months\u0026mdash;a duration significantly exceeding the median survival reported in existing series [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. We also review current evidence and propose a rational strategy for high-risk cases.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003cp\u003eA 68-year-old male presented with a two-week history of dull, non-specific epigastric pain and mild fatigue. He had no significant past medical history and denied weight loss, fever, or respiratory symptoms. Initial laboratory tests were unremarkable except for a slightly elevated D-dimer level. Contrast-enhanced chest computed tomography (CT) revealed a well-defined, heterogeneous mass measuring approximately 5.2 \u0026times; 4.1 cm in the left posterior mediastinum, adjacent to the thoracic vertebrae and compressing the descending aorta (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). No distant metastases or enlarged lymph nodes were identified.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eGiven the suspicion of a neurogenic tumor or other primary mediastinal neoplasm, the patient underwent complete resection via video-assisted thoracoscopic surgery (VATS) on August 17, 2023. Intraoperatively, the tumor was encapsulated and easily dissected from surrounding structures without macroscopic residual disease. Final pathological examination demonstrated sheets and nests of epithelioid cells with abundant eosinophilic cytoplasm, vesicular nuclei, and frequent mitotic figures. Immunohistochemical staining showed diffuse positivity for CD31, CD34, and FLI-1, (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eA-D)confirming the diagnosis of epithelioid angiosarcoma (EA) arising in the posterior mediastinum.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe patient recovered uneventfully and was discharged on postoperative day 5. However, just three weeks after surgery (September 7, 2023), he returned with worsening left-sided chest pain and progressive dyspnea. Repeat chest CT revealed a newly developed moderate left pleural effusion with hemorrhagic characteristics (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eA-B). A chest tube was inserted, yielding over 1100 mL/day of bloody fluid. Cytological analysis of the pleural fluid was negative for malignant cells, but serial hemoglobin levels showed progressive decline, raising concern for active intrathoracic bleeding.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eDue to clinical deterioration and radiographic progression, an emergency thoracotomy was performed on September 20, 2023. Intraoperative findings included thickened parietal pleura near the previous surgical bed and multiple nodular implants over the visceral pleura, highly suggestive of local recurrence with pleural dissemination (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003eA\u0026ndash;B). Frozen section confirmed recurrent epithelioid angiosarcoma. No gross residual tumor was left behind after extensive pleurectomy.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eGiven the aggressive early relapse, a multimodal salvage therapy strategy was initiated. The regimen consisted of systemic chemotherapy with paclitaxel (80 mg/m\u0026sup2; intravenously on days 1, 8, and 15 every 4 weeks) for six cycles, combined with intrapleural cisplatin instillation (40 mg weekly for two doses) administered via the indwelling chest drain to target residual microscopic disease and control malignant effusion.\u003c/p\u003e \u003cp\u003eThe patient tolerated treatment well with manageable side effects (grade 1 neutropenia and transient nausea). Follow-up imaging at 3, 6, and 12 months post-treatment initiation showed gradual resolution of pleural effusion and no evidence of new lesions. At the last follow-up in May 2025, the patient remained alive with no signs of disease progression more than 20 months after recurrence, maintaining an Eastern Cooperative Oncology Group performance status (ECOG PS) of 1 and good quality of life.​ The overall clinical timeline is illustrated in Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003ePrimary mediastinal epithelioid angiosarcoma is a diagnostic and therapeutic challenge due to its rarity and nonspecific clinical presentation [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. Our case is notable for several reasons:\u003c/p\u003e \u003cp\u003eFirst, the initial manifestation as \u003cb\u003eisolated upper abdominal pain\u003c/b\u003e led to a diagnostic delay, highlighting that deep thoracic tumors may refer pain to distant sites and mimic intra-abdominal pathology [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. This underscores the importance of including mediastinal imaging in the workup of unexplained chronic abdominal complaints, especially in older adults.\u003c/p\u003e \u003cp\u003eSecond, despite complete VATS resection\u0026mdash;a minimally invasive approach increasingly adopted for posterior mediastinal masses [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]\u0026mdash;the tumor recurred within \u003cb\u003eone month\u003c/b\u003e, indicating highly aggressive biology. Such early relapse is uncommon even among sarcomas and suggests micrometastatic spread at diagnosis [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThird, rather than opting for palliative care or single-agent therapy, we implemented an \u003cb\u003eintensified multimodal salvage strategy\u003c/b\u003e combining systemic paclitaxel and localized cisplatin perfusion. While paclitaxel has demonstrated efficacy in cutaneous and visceral angiosarcomas [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e], its use in mediastinal EA is scarcely reported. Similarly, intrapleural chemotherapy is typically reserved for symptom control in malignant effusions [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e], but here it likely contributed to local disease containment.\u003c/p\u003e \u003cp\u003eOur outcome\u0026mdash;\u003cb\u003eover 20 months of survival after recurrence\u003c/b\u003e\u0026mdash;exceeds the typical prognosis of mediastinal EA, where median survival ranges from 6 to 12 months [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Only a few case reports describe long-term survivors, usually following aggressive multimodal interventions [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe mechanism underlying the success may involve synergistic effects: systemic therapy controls occult metastases, while intrapleural delivery achieves high local drug concentration with minimal systemic exposure [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. This approach aligns with emerging trends in sarcoma management emphasizing combined modality regimens tailored to individual risk profiles.\u003c/p\u003e \u003cp\u003eNonetheless, limitations exist. We did not perform genetic profiling (e.g., \u003cem\u003eMYC\u003c/em\u003e amplification, common in radiation-associated angiosarcoma), nor did we use targeted agents or immunotherapy, which are being explored in advanced sarcomas [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Future studies should investigate biomarkers predictive of response to taxanes or platinum agents in EA.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThis case demonstrates that even in the setting of early recurrence following resection of primary posterior mediastinal epithelioid angiosarcoma, a proactive multimodal salvage strategy incorporating systemic paclitaxel and intrapleural cisplatin can lead to durable disease control and long-term survival. We advocate for heightened awareness of atypical presentations, early use of advanced imaging, and personalized, aggressive treatment approaches in managing these rare and lethal tumors.\u003c/p\u003e "},{"header":"Declarations","content":"\u003cp\u003e \u003ch2\u003eEthics approval and consent to participate\u003c/h2\u003e \u003cp\u003eAll clinical diagnoses and treatments in this article were in accordance with China\u0026rsquo;s national guidelines for diagnosis and treatment and in compliance with the requirements of the Hospital Ethics Committee.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eConsent for publication\u003c/strong\u003e \u003cp\u003eWritten informed consent was obtained from the patient for the publication of this case report and any accompanying images.\u003c/p\u003e \u003ch2\u003eCompeting interests\u003c/h2\u003e \u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cb\u003eDeclarations of Generative AI and AI-assisted technologies in the writing process\u003c/b\u003e During the preparation of this work, the authors used large language models for language editing and proofreading to improve the readability of the manuscript. After using this tool, the authors reviewed and edited the content as needed and take full responsibility for the content of the publication.\u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eThis work was supported by the Yunnan Provincial Innovative Research Team for Thoracic Tumor Prevention and Control (Grant No. 202405AS350015), the Yunnan Thoracic Surgery Doctoral Supervisor Team, the Development of a Precision Prevention and Full-Cycle Intelligent Management System for Regionally Prevalent Lung Cancer in Yunnan (Grant No. 202303AC100203), the Expert Workstation of Dr. Li Yin (Grant No. 202405AF140055), and the Joint Basic Research Program of Yunnan Provincial Department of Science and Technology and Kunming Medical University (Grant No. 202401AY07000-369).\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eS.X. and S.W. drafted the manuscript and performed the literature review. X.S. and G.L. participated in the clinical management and prepared the figures. L.Y. performed the surgery, supervised the study, and critically revised the manuscript. All authors read and approved the final manuscript.\u003c/p\u003e\u003ch2\u003eData Availability\u003c/h2\u003e\u003cp\u003eThe datasets used during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eMirza AN, Berg JW, Huvos AG, et al. Angiosarcoma: a report of 67 cases and review of the literature. 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Ann Oncol. 2012;23(10):2708\u0026ndash;13. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1093/annonc/mds093\u003c/span\u003e\u003cspan address=\"10.1093/annonc/mds093\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"journal-of-cardiothoracic-surgery","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"jcts","sideBox":"Learn more about [Journal of Cardiothoracic Surgery](http://cardiothoracicsurgery.biomedcentral.com)","snPcode":"13019","submissionUrl":"https://submission.nature.com/new-submission/13019/3","title":"Journal of Cardiothoracic Surgery","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"mediastinal tumor, epithelioid angiosarcoma, multimodal therapy, intrapleural infusion, case report","lastPublishedDoi":"10.21203/rs.3.rs-8522612/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8522612/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eWe report a rare case of primary posterior mediastinal epithelioid angiosarcoma (EA) in a 68-year-old male who presented with atypical upper abdominal pain. Contrast-enhanced chest computed tomography revealed a left posterior mediastinal mass, and histopathology with immunohistochemistry (CD31+, CD34+, FLI-1+) confirmed the diagnosis. The patient underwent complete resection via video-assisted thoracoscopic surgery (VATS). However, within one month, he developed symptomatic hemorrhagic pleural effusion, and repeat exploration confirmed local recurrence with pleural metastasis. Given the aggressive nature of EA and early relapse, a multimodal salvage regimen was initiated, consisting of systemic paclitaxel (80 mg/m\u0026sup2; on days 1, 8, 15 every 4 weeks, six cycles) and intrapleural cisplatin instillation (40 mg weekly for two doses). Remarkable clinical response was achieved, with resolution of pleural effusion and sustained disease control. The patient has remained alive with good quality of life for over 20 months post-recurrence. This case highlights that even in rapidly recurrent mediastinal EA, intensive multimodal therapy can achieve long-term survival. Our experience supports the integration of systemic chemotherapy with locoregional control strategies in managing aggressive sarcomas.\u003c/p\u003e","manuscriptTitle":"Primary Posterior Mediastinal Epithelioid Angiosarcoma Presenting as Epigastric Pain: Long-Term Survival Achieved via Multimodal Salvage Therapy after Early Recurrence","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-02-13 01:13:30","doi":"10.21203/rs.3.rs-8522612/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2026-03-24T16:20:51+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-03-06T02:33:50+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-02-12T19:15:57+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"96980199179502240600639606038387562408","date":"2026-02-10T09:55:23+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"257540123701574630167971327944259049566","date":"2026-02-09T14:18:09+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2026-02-09T02:56:22+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"105834629682239277721157049491810485250","date":"2026-02-08T22:04:12+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"154589904596433708374956199825812215634","date":"2026-02-08T20:46:39+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2026-02-08T18:50:56+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2026-01-28T05:36:25+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2026-01-28T05:33:44+00:00","index":"","fulltext":""},{"type":"submitted","content":"Journal of Cardiothoracic Surgery","date":"2026-01-05T14:36:04+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"journal-of-cardiothoracic-surgery","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"jcts","sideBox":"Learn more about [Journal of Cardiothoracic Surgery](http://cardiothoracicsurgery.biomedcentral.com)","snPcode":"13019","submissionUrl":"https://submission.nature.com/new-submission/13019/3","title":"Journal of Cardiothoracic Surgery","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"5a0146c6-50ce-4c00-8f0c-d3ebb4bfe8ee","owner":[],"postedDate":"February 13th, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2026-04-27T16:05:46+00:00","versionOfRecord":{"articleIdentity":"rs-8522612","link":"https://doi.org/10.1186/s13019-026-04130-9","journal":{"identity":"journal-of-cardiothoracic-surgery","isVorOnly":false,"title":"Journal of Cardiothoracic Surgery"},"publishedOn":"2026-04-24 16:00:01","publishedOnDateReadable":"April 24th, 2026"},"versionCreatedAt":"2026-02-13 01:13:30","video":"","vorDoi":"10.1186/s13019-026-04130-9","vorDoiUrl":"https://doi.org/10.1186/s13019-026-04130-9","workflowStages":[]},"version":"v1","identity":"rs-8522612","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8522612","identity":"rs-8522612","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}