Journal Information
Vol. 61. Issue 9.
Pages 536-543 (September 2025)
Share
Download PDF
More article options
Vol. 61. Issue 9.
Pages 536-543 (September 2025)
Original Article
Clinical Features of Primary Pulmonary Artery Sarcoma: A Systematic Review and Pooled Analysis
Visits
1878
Fumihiro Kashizakia,
, Hao Chena, Nanami Tsuchiyaa, Sachiko Matsumotoa, Reeko Osadaa, Atsushi Miyasakaa, Mai Kanekoa, Akitomo Kikuchib, Yui Kojimac, Kentaro Yumotoa, Naoki Hashiyamad, Hiroyuki Osawab, Harumi Koizumia, Makoto Mod, Kenichi Takahashia, Takeshi Kanekoe
a Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
b Department of General Thoracic Surgery, Yokohama Minami Kyosai Hospital, Yokohama, Japan
c Department of Pathology, Yokohama Minami Kyosai Hospital, Yokohama, Japan
d Department of Cardiovascular Surgery, Yokohama Minami Kyosai Hospital, Yokohama, Japan
e Department of Respiratory Medicine, Yokohama City University Hospital, Yokohama, Japan
Podcast
This item has received
Article information
Abstract
Full Text
Bibliography
Download PDF
Statistics
Figures (5)
Show moreShow less
Tables (2)
Table 1. Baseline Clinical Characteristics of PPAS.
Tables
Table 2. Treatment of PPAS.
Tables
Show moreShow less
Additional material (3)
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.

Keywords:
Primary pulmonary artery sarcoma
Multi-detector row computed tomography
18F-FDG PET/CT
Pulmonary thromboembolism
Overall survival
Review
Abbreviations:
PPAS
PAIS
MDCT
18F-FDG PET/CT
EBUS-TBNA
ECGF
PRISMA
ROB
NOS
RVOT
IQR
OS
HR
BNP
MRI
SUVmax
PTE
TBB
AI
TNM
MDM2
PDGFRA
EGFR
CDKN2A
Graphical abstract

Article

These are the options to access the full texts of the publication Archivos de Bronconeumología
Member
If you are a member of SEPAR:
  • Go to >>>SEPAR<<< website and sign in.
Subscriber
Subscriber

If you already have your login data, please click here .

If you have forgotten your password you can you can recover it by clicking here and selecting the option “I have forgotten my password”
Purchase
Purchase article

Purchasing article the PDF version will be downloaded

Purchase now
Contact
Phone for subscriptions and reporting of errors
From Monday to Friday from 9 a.m. to 6 p.m. (GMT + 1) except for the months of July and August which will be from 9 a.m. to 3 p.m.
Calls from Spain
932 415 960
Calls from outside Spain
+34 932 415 960
Email
Archivos de Bronconeumología
Article options
Tools

Are you a health professional able to prescribe or dispense drugs?