Royal Papworth Hospital, Papworth Road, Trumpington, Cambridge, CB2 0AY, UK.
J Cardiothorac Surg. 2024 May 14;19(1):288. doi: 10.1186/s13019-024-02700-3.
Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.
肺动脉肉瘤（PAS）是一种罕见的侵袭性肿瘤，主要发生在肺动脉干。我们报告了一例累及肺动脉干壁和瓣膜的 PAS 病例，其特征为均匀的壁增厚，这是该肿瘤的一种非典型影像学表现。一名 63 岁男性因进行性加重的呼吸症状就诊。CTPA 显示肺动脉内有低密度充盈缺损，而 PET 扫描显示肺动脉干摄取增加，同时伴有血沉升高，提示为肺动脉炎。超声心动图显示右心室肥厚和肺动脉瓣狭窄。激素治疗反应不佳，症状恶化。他被转介至另一家医院，被诊断为 PAS。他接受了肺动脉血栓内膜切除术和肺动脉瓣置换术。术后组织病理学检查证实了诊断。PAS 较为罕见，常被误诊。手术切除不能治愈，但与化疗联合可延长生存时间。
