Clinical Features of Primary Pulmonary Artery Sarcoma: A Systematic Review and Pooled Analysis.

IF 8.7 3区医学 Q1 RESPIRATORY SYSTEM Archivos De Bronconeumologia Pub Date : 2024-12-20 DOI:10.1016/j.arbres.2024.12.012

Fumihiro Kashizaki, Hao Chen, Nanami Tsuchiya, Sachiko Matsumoto, Reeko Osada, Atsushi Miyasaka, Mai Kaneko, Akitomo Kikuchi, Yui Kojima, Kentaro Yumoto, Naoki Hashiyama, Hiroyuki Osawa, Harumi Koizumi, Makoto Mo, Kenichi Takahashi, Takeshi Kaneko

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Abstract

Objectives: Primary pulmonary artery sarcoma (PPAS) is a rare disease with unclear clinical manifestations. Advances in imaging devices have improved diagnostic capabilities, potentially affecting clinical characteristics and overall survival (OS); however, details remain unclear. This study conducted a pooled analysis of case reports and series to analyse the clinical characteristics and OS of PPAS in the era of advanced medical devices.

Methods: Data were sourced from PubMed and CINAHL, focusing on studies published between 1 January 2014 and 31 December 2023. The study included patients diagnosed with PPAS, with extracted data covering demographics, diagnosis, treatments, and survival.

Results: Overall, 643 patients were included (mean age: 52.6±13.1 years; 50.4% were female). Initially, 70.6% were diagnosed with pulmonary thromboembolism (PTE), and 15.4% were suspected of having PPAS. Among these, 93.9% and 55.2% showed suggestive findings on computed tomography (CT)-integrated positron emission tomography with 2-deoxy-2-18F-fluoro-d-glucose (18F-FDG PET/CT) and CT, respectively, with 98.2% confirmed before death. The right main pulmonary artery was the most affected site on CT (72.3%). Surgery and chemotherapy were performed in 81.4% and 66.4% of patients, respectively. The median OS was 31 months, with surgery extending OS across all stages and chemotherapy benefiting stages III-IV. Longer OS was achieved in patients who underwent complete surgical resection.

Conclusions: 18F-FDG PET/CT and multi-detector-row CT can differentiate PTE from PPAS. These medical devices may contribute to improved OS.

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原发性肺动脉肉瘤的临床特征：系统回顾和汇总分析。

目的：原发性肺动脉肉瘤（PPAS）是一种临床表现不明确的罕见疾病。成像设备的进步提高了诊断能力，可能会影响临床特征和总生存期（OS）；然而，细节尚不清楚。本研究通过病例报告和系列资料的汇总分析，分析先进医疗器械时代PPAS的临床特点和OS。方法：数据来源于PubMed和CINAHL，集中于2014年1月1日至2023年12月31日发表的研究。该研究包括被诊断为PPAS的患者，提取的数据包括人口统计学、诊断、治疗和生存。结果：共纳入643例患者(平均年龄：52.6±13.1岁；50.4%为女性)。最初，70.6%被诊断为肺血栓栓塞（PTE）， 15.4%被怀疑患有PPAS。其中93.9%和55.2%分别在2-脱氧-2- 18f -氟-d-葡萄糖（18F-FDG PET/CT）和CT上显示暗示性发现，其中98.2%在死亡前确诊。CT显示右肺主干病变最多（72.3%）。手术和化疗分别占81.4%和66.4%。中位生存期为31个月，手术延长了所有阶段的生存期，化疗使III-IV期受益。接受完全手术切除的患者获得了更长的OS。结论：18F-FDG PET/CT及多排CT可鉴别PTE与PPAS。这些医疗设备可能有助于改进操作系统。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Archivos De Bronconeumologia Medicine-Pulmonary and Respiratory Medicine

CiteScore

3.50

自引率

17.50%

发文量

330

审稿时长

14 days

期刊介绍： Archivos de Bronconeumologia is a scientific journal that specializes in publishing prospective original research articles focusing on various aspects of respiratory diseases, including epidemiology, pathophysiology, clinical practice, surgery, and basic investigation. Additionally, the journal features other types of articles such as reviews, editorials, special articles of interest to the society and editorial board, scientific letters, letters to the editor, and clinical images. Published monthly, the journal comprises 12 regular issues along with occasional supplements containing articles from different sections. All manuscripts submitted to the journal undergo rigorous evaluation by the editors and are subjected to expert peer review. The editorial team, led by the Editor and/or an Associate Editor, manages the peer-review process. Archivos de Bronconeumologia is published monthly in English, facilitating broad dissemination of the latest research findings in the field.