Momand Ghulamhadi, van der Laan Maarten J, Zeebregts Clark J, Tielliu Ignace F J
Department of Surgery (Division of Vascular Surgery), University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
J Vasc Surg Cases Innov Tech. 2025 Jun 13;11(5):101878. doi: 10.1016/j.jvscit.2025.101878. eCollection 2025 Oct.
Primary aortic angiosarcoma is a rare and aggressive malignancy arising from endothelial cells of blood vessels and has a poor prognosis. We report 21 relevant cases from the literature, summarizing the number of reported cases without analyzing treatment outcomes or prognostic data. Additionally, we report our own case of a 75-year-old male who presented with fever, abdominal pain, and leg discomfort 10 years after endovascular aneurysm repair (EVAR) for an abdominal aortic aneurysm. Initial imaging suggested an infected EVAR, leading to explantation and reconstruction. Despite antibiotic treatment, negative cultures and surgical intervention, the patient's condition continued to deteriorate. A biopsy of cervical lymph nodes revealed a high-grade angiosarcoma. These cases highlight the diagnostic challenges of primary aortic angiosarcoma, especially in patients with prior EVAR, and emphasize the importance of considering rare malignancies in cases when a patient deteriorates despite therapy or does not show the expected treatment response.
原发性主动脉血管肉瘤是一种罕见的侵袭性恶性肿瘤，起源于血管内皮细胞，预后较差。我们报告了文献中的21例相关病例，总结了报告病例数，未分析治疗结果或预后数据。此外，我们报告了我们自己的一例病例，一名75岁男性，在接受腹主动脉瘤血管内动脉瘤修复术（EVAR）10年后出现发热、腹痛和腿部不适。最初的影像学检查提示为感染性EVAR，导致进行了移植物取出和重建手术。尽管进行了抗生素治疗、培养结果为阴性且进行了手术干预，但患者的病情仍继续恶化。颈部淋巴结活检显示为高级别血管肉瘤。这些病例突出了原发性主动脉血管肉瘤的诊断挑战，尤其是在有既往EVAR史的患者中，并强调了在患者尽管接受治疗仍病情恶化或未表现出预期治疗反应的情况下考虑罕见恶性肿瘤的重要性。
