Campana Federica, Nardin Matteo, Coppini Anna, Muiesan Maria Lorenza
Department of Clinical & Experimental Sciences, University of Brescia & Postgraduate School of Internal Medicine University of Brescia, Piazzale Spedali Civili 1, Brescia, Italy.
2a Medicina Generale ASST Spedali Civili, Piazzale Spedali Civili 1, Brescia, Italy.
Eur Heart J Case Rep. 2019 Sep 1;3(3). doi: 10.1093/ehjcr/ytz114.
Aortic thrombosis represents a consequence of atherosclerotic disease. In few cases, it can be secondary to large vessel or infective vasculitis. More rarely, aortic thrombosis is the manifestation of a primary malignant neoplasm of the aortic wall. Aortic angiosarcoma is a rare tumour, its clinical presentation is often non-specific and associated signs and symptoms may vary greatly. An early diagnosis is difficult to reach and the presence of metastatic disease is not uncommon at the time of diagnosis. The prognosis is poor overall.
We report the case of a female patient who presented to her GP because of fatigue, hyporexia, weight-loss, and anaemia. An ultrasound of the abdomen showed two small pancreatic lesions, confirmed and described as benign cystic pancreatic lesions on computed tomography (CT) imaging; an incidental thrombus in the superior mesenteric artery was also found on CT imaging. The thoracic CT identified a large thrombotic lesion in the descending thoracic aorta with significant narrowing of the aortic lumen and confirmed the presence of an osteolytic bone lesion on the VIII right rib, in the absence of atherosclerotic disease. Signs of increased metabolic activity in the aortic lumen and in the VIII posterior right rib were shown at a subsequent positron emission tomography. A CT-guided biopsy of the bone lesion was performed and at histology the diagnosis of metastatic angiosarcoma of the aortic wall was made.
Aortic angiosarcoma is a rare cause of aortic thrombosis, to be taken into consideration in a patient with thrombotic lesions of the aorta in the absence of atherosclerotic disease. The differential diagnosis is difficult because of clinical presentation and radiological features similar to those of inflammatory aortic disease. In our case, the final diagnosis of angiosarcoma was made only by a biopsy of a bone metastatic lesion.
主动脉血栓形成是动脉粥样硬化疾病的一种后果。在少数情况下，它可能继发于大血管或感染性血管炎。更罕见的是，主动脉血栓形成是主动脉壁原发性恶性肿瘤的表现。主动脉血管肉瘤是一种罕见的肿瘤，其临床表现通常不具有特异性，相关的体征和症状可能差异很大。早期诊断很难实现，并且在诊断时出现转移性疾病并不罕见。总体预后较差。
我们报告了一名女性患者的病例，该患者因疲劳、食欲减退、体重减轻和贫血就诊于全科医生。腹部超声显示胰腺有两个小病变，计算机断层扫描（CT）成像证实并描述为良性胰腺囊性病变；CT成像还发现肠系膜上动脉有一个偶然发现的血栓。胸部CT在降主动脉发现一个大的血栓性病变，主动脉腔明显狭窄，并证实右侧第八肋骨存在溶骨性骨病变，且无动脉粥样硬化疾病。随后的正电子发射断层扫描显示主动脉腔和右侧第八后肋骨有代谢活性增加的迹象。对骨病变进行了CT引导下活检，组织学检查诊断为主动脉壁转移性血管肉瘤。
主动脉血管肉瘤是主动脉血栓形成的罕见原因，在没有动脉粥样硬化疾病的主动脉血栓性病变患者中应予以考虑。由于临床表现和放射学特征与炎症性主动脉疾病相似，鉴别诊断困难。在我们的病例中，仅通过对骨转移病变的活检才做出血管肉瘤的最终诊断。
