Guarnieri Gianluca, Briguglia Daniele, Conte Edoardo, Andreini Daniele
Department of Biomedical and Clinical Sciences, University of Milan, via Festa del Perdono, Milan 20122, Italy.
Division of University Cardiology, IRCCS Ospedale Galeazzi Sant'Ambrogio, via Belgioioso, Milan 20157, Italy.
Eur Heart J Case Rep. 2025 Apr 25;9(5):ytaf207. doi: 10.1093/ehjcr/ytaf207. eCollection 2025 May.
Pulmonary artery angiosarcoma is a rare and highly malignant tumour. Its clinical presentation often mimics common pulmonary disorders such as pulmonary embolism or pneumonia, which frequently leads to misdiagnosis. Due to its aggressive nature and typical diagnostic delay, prognosis is generally poor, with survival often limited to a few months post-diagnosis.
A 70-year-old female with a history of hypertension and no previous cardiac issues presented with progressive exertional dyspnoea unresponsive to anticoagulation after a diagnosis of pulmonary embolism. A chest computed tomography (CT) initially suggested a pulmonary embolism with an endoluminal filling defect in the right pulmonary artery. However, further investigation revealed a mass in the right pulmonary artery with a subsequent obliteration of the perihilar fat spaces, leading to a diagnosis of pulmonary artery angiosarcoma.
This case underscores the importance of considering pulmonary artery angiosarcoma in differential diagnoses for persistent pulmonary symptoms, especially when imaging and clinical signs are atypical for thromboembolic disease. Early recognition and a multidisciplinary approach are essential for improving outcomes in this rare but lethal malignancy, where prompt diagnosis may extend survival significantly.
肺动脉血管肉瘤是一种罕见的高恶性肿瘤。其临床表现常类似于常见的肺部疾病，如肺栓塞或肺炎，这常常导致误诊。由于其侵袭性本质和典型的诊断延迟，预后通常较差，生存期通常仅限于诊断后几个月。
一名70岁女性，有高血压病史，既往无心脏问题，在诊断为肺栓塞后出现进行性劳力性呼吸困难，抗凝治疗无效。胸部计算机断层扫描（CT）最初提示肺栓塞，右肺动脉内有腔内充盈缺损。然而，进一步检查发现右肺动脉有肿块，随后肺门周围脂肪间隙消失，最终诊断为肺动脉血管肉瘤。
该病例强调了在持续性肺部症状的鉴别诊断中考虑肺动脉血管肉瘤的重要性，尤其是当影像学和临床体征不符合血栓栓塞性疾病的典型表现时。早期识别和多学科方法对于改善这种罕见但致命的恶性肿瘤的治疗结果至关重要，及时诊断可能显著延长生存期。
