大块原发性肺动脉横纹肌肉瘤1例报告。

IF 1.6 Q2 MEDICINE, GENERAL & INTERNAL Romanian Journal of Internal Medicine Pub Date : 2024-03-23 Print Date: 2024-03-01 DOI:10.2478/rjim-2023-0032
Farid Rashidi, Eissa Bilehjani, Seyed Ali Mousavi-Aghdas, Rezayat Parvizi
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引用次数: 0

摘要

背景:肺动脉肉瘤(PAS)是一种罕见的肿瘤,引起肺循环的隐性进行性阻塞。临床表现往往难以区分慢性血栓栓塞性肺动脉高压(CTEPH)。然而,CT肺血管造影(CTPA)中不典型的异质充盈缺陷的表现应引起进一步的研究。病例介绍:一名健康的年轻男性,自一年前以来出现大量咯血,急性呼吸窘迫和进行性用力呼吸困难。超声心动图显示严重的右心室功能障碍和极可能的肺动脉高压。CTPA显示广泛的充盈缺陷,其外观与PAS有关。由于休息时晕厥发作,患者接受了紧急肺动脉内膜切除术(PEA)。结果切除了一个巨大的树状肿瘤。术后,再灌注损伤和难治性肺水肿需要体外膜氧合(ECMO)。不幸的是,ECMO并发大量溶血和急性肾损伤。病人死于多器官衰竭。通过组织分析确定胚胎性横纹肌肉瘤的诊断。讨论:不幸的是,病人并没有及时处理他日益恶化的呼吸困难。PASs不应被误认为是血栓,应避免抗凝。由于病情紧急,无法进行活检和组织诊断。同样,新辅助化疗也不可行。术后出现再灌注损伤和肺水肿,需进行ECMO。这种并发症应在术前预料到。需要更多关于PASs的数据,以便为管理建立共识。
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Massive primary pulmonary artery rhabomyosarcoma: A case report.

Background: Pulmonary artery sarcomas (PAS) are rare tumours causing an insidiously progressive obstruction of the pulmonary circulation. The clinical presentation is often indistinguishable from chronic thromboembolic pulmonary hypertension (CTEPH). However, the atypical appearance of a heterogeneous filling defect in CT pulmonary angiography (CTPA) should prompt further investigation.

Case presentation: A previously healthy young man presented with massive haemoptysis, acute respiratory distress, and progressive exertional dyspnea since the year before. Echocardiography demonstrated severe right ventricular dysfunction and highly probable pulmonary hypertension. CTPA revealed an extensive filling defect with an appearance concerning PAS. Due to syncopal episodes at rest, the patient underwent urgent pulmonary artery endarterectomy (PEA). A massive tree-like tumour was excised as a result. Post-operatively, reperfusion injury and refractory pulmonary oedema mandated extracorporeal membrane oxygenation (ECMO). Unfortunately, ECMO was complicated with massive haemolysis and acute kidney injury. The patient succumbed to multi-organ failure. Through tissue analysis established a diagnosis of embryonal rhabdomyosarcoma.

Discussion: Unfortunately, the patient had not reached out for his worsening dyspnea. PASs should not be mistaken for a thrombus and anticoagulation should be avoided. The urgent condition precluded biopsy and tissue diagnosis. Similarly, neoadjuvant chemotherapy was not feasible. Post-operatively, reperfusion injury and pulmonary oedema ensued, which mandated ECMO. This complication should be anticipated preoperatively. There is a need for more data on PASs to establish a consensus for management.

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来源期刊
Romanian Journal of Internal Medicine
Romanian Journal of Internal Medicine MEDICINE, GENERAL & INTERNAL-
CiteScore
3.20
自引率
5.30%
发文量
35
审稿时长
15 weeks
期刊介绍: Romanian Journal of Physics is a journal publishing physics contributions on the following themes: •Theoretical Physics & Applied Mathematics •Nuclear Physics •Solid State Physics & Materials Science •Statistical Physics & Quantum Mechanics •Optics •Spectroscopy •Plasma & Lasers •Nuclear & Elementary Particles Physics •Atomic and Molecular Physics •Astrophysics •Atmosphere and Earth Science •Environment Protection
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