Primary Pulmonary Angiosarcoma Found Incidentally in a Complicated Patient: A Rare Case Report

IF 1.9 4区 医学 Q3 RESPIRATORY SYSTEM Clinical Respiratory Journal Pub Date : 2024-08-06 DOI:10.1111/crj.13818
Reza Basiri, Alireza Ziaei Moghaddam, Arezoo Rikhtegar, Amir Hossein Jafarian
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Abstract

Introduction

Primary pulmonary angiosarcoma (PPA) is a highly aggressive and rare malignancy originating from the endothelial cells of blood vessels in the lungs. PPA is an extremely rare subtype, with less than 30 cases reported to date. PPA is not only challenging to diagnose but also has a poor prognosis, often resulting in a high mortality rate within a year of diagnosis, regardless of the treatment approach.

Method

We present the case of a 33-year-old woman with no significant past medical history who presented with abdominal pain and was incidentally found to have a right hilar mass with pleural effusion and empyema. After undergoing surgery for a perforated gastric ulcer, her pulmonary lesions were further worked up. Despite an extensive diagnostic evaluation, including imaging, bronchoscopy, and thoracotomy, establishing a diagnosis was challenging. Ultimately, PPA was diagnosed on surgical lung biopsy, and the patient was started on pazopanib and paclitaxel chemotherapy but expired after 1 month due to multiple complications.

Conclusion

This case highlights the difficulty in diagnosing this rare tumor and its poor prognosis regardless of therapy. Greater awareness of PPA and more research are needed to improve early detection and treatment options for this deadly disease.

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并发症患者偶然发现的原发性肺血管肉瘤:罕见病例报告
简介原发性肺血管肉瘤(PPA)是一种源自肺部血管内皮细胞的高度侵袭性罕见恶性肿瘤。PPA 是一种极为罕见的亚型,迄今报道的病例不到 30 例。PPA 不仅诊断困难,而且预后较差,无论采用何种治疗方法,通常在确诊后一年内死亡率较高:我们介绍了一例 33 岁女性的病例,她既往无重大病史,因腹痛就诊,偶然发现右侧腹股沟肿块,伴有胸腔积液和肺水肿。在接受胃溃疡穿孔手术后,她的肺部病变得到了进一步检查。尽管进行了广泛的诊断评估,包括影像学检查、支气管镜检查和胸腔切开术,但确诊仍很困难。最终,手术肺活检确诊为 PPA,患者开始接受帕唑帕尼和紫杉醇化疗,但 1 个月后因多种并发症去世:本病例凸显了诊断这种罕见肿瘤的难度,以及无论采用何种治疗方法,其预后都很差。我们需要提高对PPA的认识并开展更多研究,以改善这种致命疾病的早期发现和治疗方案。
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来源期刊
Clinical Respiratory Journal
Clinical Respiratory Journal 医学-呼吸系统
CiteScore
3.70
自引率
0.00%
发文量
104
审稿时长
>12 weeks
期刊介绍: Overview Effective with the 2016 volume, this journal will be published in an online-only format. Aims and Scope The Clinical Respiratory Journal (CRJ) provides a forum for clinical research in all areas of respiratory medicine from clinical lung disease to basic research relevant to the clinic. We publish original research, review articles, case studies, editorials and book reviews in all areas of clinical lung disease including: Asthma Allergy COPD Non-invasive ventilation Sleep related breathing disorders Interstitial lung diseases Lung cancer Clinical genetics Rhinitis Airway and lung infection Epidemiology Pediatrics CRJ provides a fast-track service for selected Phase II and Phase III trial studies. Keywords Clinical Respiratory Journal, respiratory, pulmonary, medicine, clinical, lung disease, Abstracting and Indexing Information Academic Search (EBSCO Publishing) Academic Search Alumni Edition (EBSCO Publishing) Embase (Elsevier) Health & Medical Collection (ProQuest) Health Research Premium Collection (ProQuest) HEED: Health Economic Evaluations Database (Wiley-Blackwell) Hospital Premium Collection (ProQuest) Journal Citation Reports/Science Edition (Clarivate Analytics) MEDLINE/PubMed (NLM) ProQuest Central (ProQuest) Science Citation Index Expanded (Clarivate Analytics) SCOPUS (Elsevier)
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