Pulmonary artery sarcoma presenting as chronic thromboembolic pulmonary hypertension (CTEPH).

Fotini Ampatzidou, Odysseas Drosos, Athanasios Madesis, George Drossos
{"title":"Pulmonary artery sarcoma presenting as chronic thromboembolic pulmonary hypertension (CTEPH).","authors":"Fotini Ampatzidou, Odysseas Drosos, Athanasios Madesis, George Drossos","doi":"10.5114/kitp.2023.129538","DOIUrl":null,"url":null,"abstract":"Address for correspondence: Dr. Fotini Ampatzidou, Department of Cardiac Surgery, ICU, General Hospital “G. Papanikolaou”, Exohi, 57010, Thessaloniki, Greece, e-mail: fampatzidou@gmail.com Received: 27.12.2022, accepted: 17.03.2023. Pulmonary artery sarcoma (PAS) is an extremely rare neoplasm arising from mesenchymal cells of the pulmonary artery [1]. PAS was first described in 1923, its incidence is about 0.001% to 0.03% and it is characterized by a very poor prognosis [2]. Early and correct diagnosis is crucial. Unfortunately misdiagnosis is quite common due to overlapping clinical characteristics with pulmonary embolism (PE) or chronic thromboembolic pulmonary hypertension (CTEPH), leading not only to delayed therapeutic management but also to unnecessary (and associated with hemorrhagic risks) thrombolytic and anticoagulation therapy [3]. A 68-year-old woman was referred to our Cardiothoracic Department with a case of chronic pulmonary thromboembolic disease in order to undergo pulmonary endarterectomy. She had a history of dyspnea one year ago with gradual deterioration during the last 3 months. A few days before, she was admitted to the Pneumonology Department suffering from thoracic pain. D-dimer levels were mildly elevated. She underwent a chest computed tomography (CT) scan which revealed an inhomogeneous mass arising into the medial bronchopulmonary segment with irregular borders. Triplex vascular ultrasound was performed twice and was negative for deep venous thrombosis. CT pulmonary angiography was suggestive for pulmonary embolism based on the following signs: filling defects in right main bronchus expanded peripherally to medial and lower lobe. Ventilation/perfusion lung scan showed normal ventilation with complete absence of perfusion of the right lung. Dilated right ventricle and estimated systolic pulmonary artery of 39 mm Hg were the main echocardiographic findings. She underwent an elective cardiothoracic surgical procedure with the use of a cardiopulmonary bypass under deep hypothermia (lowest 19°C). Intraoperatively, material from the right pulmonary artery was obtained and examined in frozen sections (Figure 1). The main finding is a mass protruding in the lumen of the pulmonary artery and filling its branches in a mold-like manner (Figure 2). The frozen sections (Figure 3) demonstrated a malignant neoplasm with sarcomatous features. It consisted of highly pleomorphic cells with evident mitotic activity, in an edematous or slightly myxoid background. The cells were either spindle-shaped with enlarged, elongated nuclei, or round to oval-shaped with vesicular nuclei. There were also scattered multi-nucleated cells with lobular nuclei. Subsequently, a right pneumonectomy was performed. Duration of mechanical ventilation was 20 hours and ICU stay lasted 3 days. The patient was discharged on the 6th postoperative day without complications. She underwent chemotherapy and survived 11 months. Intimal sarcoma of the pulmonary artery was first described by Moritz Mandelstamm in 1923 [4]. The true incidence of pulmonary artery intimal sarcoma is unknown, because it is most often misdiagnosed. A high level of suspicion is required due to resemblance in clinical presentation to acute or chronic pulmonary embolism (dyspnea, chest pain, cough, hemoptysis). Hemodynamic instability is suggestive for PE while IPAS is characterized by more long standing symptoms. Diagnosis is based on contemPulmonary artery sarcoma presenting as chronic thromboembolic pulmonary hypertension (CTEPH)","PeriodicalId":49945,"journal":{"name":"Kardiochirurgia I Torakochirurgia Polska","volume":"20 2","pages":"129-131"},"PeriodicalIF":0.6000,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d9/98/KITP-20-51113.PMC10410642.pdf","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Kardiochirurgia I Torakochirurgia Polska","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.5114/kitp.2023.129538","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"SURGERY","Score":null,"Total":0}
引用次数: 0

Abstract

Address for correspondence: Dr. Fotini Ampatzidou, Department of Cardiac Surgery, ICU, General Hospital “G. Papanikolaou”, Exohi, 57010, Thessaloniki, Greece, e-mail: fampatzidou@gmail.com Received: 27.12.2022, accepted: 17.03.2023. Pulmonary artery sarcoma (PAS) is an extremely rare neoplasm arising from mesenchymal cells of the pulmonary artery [1]. PAS was first described in 1923, its incidence is about 0.001% to 0.03% and it is characterized by a very poor prognosis [2]. Early and correct diagnosis is crucial. Unfortunately misdiagnosis is quite common due to overlapping clinical characteristics with pulmonary embolism (PE) or chronic thromboembolic pulmonary hypertension (CTEPH), leading not only to delayed therapeutic management but also to unnecessary (and associated with hemorrhagic risks) thrombolytic and anticoagulation therapy [3]. A 68-year-old woman was referred to our Cardiothoracic Department with a case of chronic pulmonary thromboembolic disease in order to undergo pulmonary endarterectomy. She had a history of dyspnea one year ago with gradual deterioration during the last 3 months. A few days before, she was admitted to the Pneumonology Department suffering from thoracic pain. D-dimer levels were mildly elevated. She underwent a chest computed tomography (CT) scan which revealed an inhomogeneous mass arising into the medial bronchopulmonary segment with irregular borders. Triplex vascular ultrasound was performed twice and was negative for deep venous thrombosis. CT pulmonary angiography was suggestive for pulmonary embolism based on the following signs: filling defects in right main bronchus expanded peripherally to medial and lower lobe. Ventilation/perfusion lung scan showed normal ventilation with complete absence of perfusion of the right lung. Dilated right ventricle and estimated systolic pulmonary artery of 39 mm Hg were the main echocardiographic findings. She underwent an elective cardiothoracic surgical procedure with the use of a cardiopulmonary bypass under deep hypothermia (lowest 19°C). Intraoperatively, material from the right pulmonary artery was obtained and examined in frozen sections (Figure 1). The main finding is a mass protruding in the lumen of the pulmonary artery and filling its branches in a mold-like manner (Figure 2). The frozen sections (Figure 3) demonstrated a malignant neoplasm with sarcomatous features. It consisted of highly pleomorphic cells with evident mitotic activity, in an edematous or slightly myxoid background. The cells were either spindle-shaped with enlarged, elongated nuclei, or round to oval-shaped with vesicular nuclei. There were also scattered multi-nucleated cells with lobular nuclei. Subsequently, a right pneumonectomy was performed. Duration of mechanical ventilation was 20 hours and ICU stay lasted 3 days. The patient was discharged on the 6th postoperative day without complications. She underwent chemotherapy and survived 11 months. Intimal sarcoma of the pulmonary artery was first described by Moritz Mandelstamm in 1923 [4]. The true incidence of pulmonary artery intimal sarcoma is unknown, because it is most often misdiagnosed. A high level of suspicion is required due to resemblance in clinical presentation to acute or chronic pulmonary embolism (dyspnea, chest pain, cough, hemoptysis). Hemodynamic instability is suggestive for PE while IPAS is characterized by more long standing symptoms. Diagnosis is based on contemPulmonary artery sarcoma presenting as chronic thromboembolic pulmonary hypertension (CTEPH)

Abstract Image

Abstract Image

Abstract Image

查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
肺动脉肉瘤表现为慢性血栓栓塞性肺动脉高压(CTEPH)。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 去求助
来源期刊
CiteScore
0.90
自引率
14.30%
发文量
44
审稿时长
6-12 weeks
期刊介绍: Polish Journal of Thoracic and Cardiovascular Surgery is a quarterly aimed at cardiologists, cardiosurgeons and thoracic surgeons. Includes the original works (experimental, research and development), illustrative and casuistical works about cardiology and cardiosurgery.
期刊最新文献
Clinicodemographic profile and outcomes of congenital diaphragmatic hernia with sac: experience of a paediatric referral centre. Comparison of the effect of chest tube diameter on drainage rate and tube performance in patients with pleural effusion. A cross-sectional study. Enhancing treatment approaches for postpneumonectomy empyema: exploring the role of video-assisted thoracic surgery. Optimal timing of percutaneous coronary intervention in elderly patients with acute ST-segment elevation myocardial infarction presenting late. Perceval sutureless bioprosthesis versus Trifecta sutured bioprosthesis for aortic valve replacement: immediate results of the Perfecta study.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1