Morimoto Kenichi, Miyasaka Shigeto, Ikeda Yosuke, Nii Rikuto, Fujiwara Yoshikazu
Department of Cardiovascular Surgery, Tottori Prefectural Central Hospital, 730 Ezu, Tottori 680-0901, Japan.
Department of Cardiovascular Surgery, Tottori Prefectural Central Hospital, 730 Ezu, Tottori 680-0901, Japan.
Int J Surg Case Rep. 2024 Feb;115:109308. doi: 10.1016/j.ijscr.2024.109308. Epub 2024 Jan 30.
Primary angiosarcoma of the aorta, particularly within the sinus of Valsalva, is uncommon, with no documented instances of primary angiosarcoma. The absence of apparent clinical manifestations in this severe condition makes it challenging to diagnose, often resulting in a poor prognosis.
A 60-year-old patient underwent procedures for fistula closure and coronary artery bypass grafting, which resulted in the rupture of an aneurysm within the sinus of Valsalva. Computed tomography examination 5 years after the procedure suggested no pathological abnormalities. Nevertheless, the patient required repeat surgery at 67 years due to the observed expansion of the sinus of Valsalva aneurysm noted during a clinical evaluation, prompted by elevated levels of inflammatory markers. Exploration of the residual aneurysmal locus within the sinus of Valsalva revealed an intraluminal thrombus devoid of any demonstrable hemodynamic access into the aneurysmal sac. Histopathological assessment of the aneurysmal wall confirmed a definitive diagnosis of primary angiosarcoma within the sinus of Valsalva. After surgery, the patient exhibited pyrexia. Magnetic resonance imaging substantiated multifocal osseous metastases, corroborated by histological analysis following a bone biopsy, confirming a diagnosis of angiosarcoma. Therefore, adjuvant chemotherapy with paclitaxel was initiated. After 1 year, a sustained state of disease stability was noted.
In this case, the need for surgical intervention, based on an expanded sinus of Valsalva aneurysm, culminated in the unanticipated detection of primary angiosarcoma.
Neoplastic etiologies may plausibly underlie the pathogenesis of aneurysm formation in cases where the etiology remains obscure in the early stages of therapeutic intervention.
主动脉原发性血管肉瘤,尤其是在主动脉瓣窦内的血管肉瘤,较为罕见,尚无原发性血管肉瘤的文献记载病例。这种严重疾病缺乏明显的临床表现,使其诊断具有挑战性,常常导致预后不良。
一名60岁患者接受了瘘管闭合和冠状动脉搭桥手术,导致主动脉瓣窦内的动脉瘤破裂。术后5年的计算机断层扫描检查未显示病理异常。然而,由于临床评估中发现主动脉瓣窦动脉瘤扩大,炎症标志物水平升高,该患者在67岁时需要再次手术。对主动脉瓣窦内残留的动脉瘤部位进行探查,发现腔内血栓,未发现任何可证明的血液动力学通道进入动脉瘤囊。对动脉瘤壁的组织病理学评估证实了主动脉瓣窦内原发性血管肉瘤的明确诊断。手术后,患者出现发热。磁共振成像证实有多发性骨转移,骨活检后的组织学分析也证实了这一点,确诊为血管肉瘤。因此,开始使用紫杉醇进行辅助化疗。1年后,病情处于持续稳定状态。
在本病例中,基于扩大的主动脉瓣窦动脉瘤而进行的手术干预最终意外发现了原发性血管肉瘤。
在治疗干预早期病因仍不明确的情况下,肿瘤病因可能是动脉瘤形成发病机制的合理基础。