Pulmonary artery intimal sarcoma presenting as lung mass

Aniza Hassan , Sarita Prabhakaran , Joanna Connor , Nicola Kingston , Sonja Klebe
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Abstract

This report describes a case of incidental lung lesions, discovered during investigations for symptomatic aortic stenosis. The chest computed tomography pulmonary angiogram (CTPA) revealed the presence of two lung masses in the background of longstanding pulmonary thromboembolic disease. Left upper lobectomy was performed and the histopathological examination of the lung masses showed a high-grade spindle cells malignancy with areas showing tumour cells intraluminally in large and small vessels causing partial and complete luminal occlusion. The Fluorescence in situ hybridization (FISH) study showed Mouse double minute 2 homolog (MDM2) amplification, which was consistent with the diagnosis of Pulmonary Artery Intimal Sarcoma (PAS). We report the rare entity of PAS presenting as a lung mass and some potential pitfalls in diagnosis.

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肺动脉内膜肉瘤表现为肺肿块
本报告描述了一例偶然的肺部病变,在症状性主动脉狭窄的调查中发现。胸部计算机断层扫描肺血管造影(CTPA)显示,在长期肺血栓栓塞疾病的背景下,存在两个肺部肿块。进行了左上肺叶切除术,肺部肿块的组织病理学检查显示为高度梭形细胞恶性肿瘤,区域显示大小血管中的肿瘤细胞,导致部分和完全管腔闭塞。荧光原位杂交(FISH)研究显示小鼠双分钟2同源物(MDM2)扩增,这与肺动脉内膜肉瘤(PAS)的诊断一致。我们报告了罕见的PAS实体表现为肺部肿块和诊断中的一些潜在陷阱。
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