title: Clinical Characteristics and Treatment Outcomes of Primary Pulmonary Artery Sarcoma in Korea.
abstract: Pulmonary artery sarcomas (PAS) are rare malignant neoplasms. Right heart failure due to tumour location is the main cause of death in PAS patients. The hemodynamic influence of PAS may effect prognosis, but this has not been proven. We aimed to identify the clinical characteristics and prognostic factors of PAS in Korea, their association with pulmonary hypertension (PH). PAS patients treated at the Asan Medical Center between 2000 and 2014 were reviewed. We examined demographic characteristics, diagnostic and treatment modalities. Potential prognostic factors were evaluated by univariate and multivariate analysis. Twenty patients were diagnosed with PAS. Ten patients were male, the median age was 54 years (range, 33-75 years). The most common symptom observed was dyspnea (65%). The most common histologic type was spindle cell sarcoma (30%). Ten patients had a presumptive diagnosis of pulmonary embolism (PE) and received anticoagulation therapy. Seventeen patients underwent surgery, but only 5 patients had complete resection. Eleven patients received post-operative treatment (chemotherapy = 3, radiotherapy = 5, chemoradiotherapy = 3). PH was observed in 12 patients before treatment and in 6 patients after treatment. Overall median survival was 24 months. Post-treatment PH was associated with poor prognosis (HR 9.501, 95% CI 1.79-50.32; P = 0.008) while chemotherapy was negatively associated with mortality (HR 0.102, 95% CI 0.013-0.826; P = 0.032) in univariate analysis. Post-treatment PH was also associated with poor prognosis in multivariate analysis (HR 5.7, 95% CI 1.08-30.91; P = 0.041). PAS patients are frequently misdiagnosed with PE in Korea. Post-treatment PH is associated with a poor prognosis.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Yunkyoung
authors.identifier: http://orcid.org/0000-0001-7364-5609
authors.initials: Y
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, Kyungpook National University Hospital, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Hyun Jung
authors.identifier: http://orcid.org/0000-0002-1878-1111
authors.initials: HJ
authors.lastname: Kim
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Heeyoung
authors.identifier: http://orcid.org/0000-0001-7109-1374
authors.initials: H
authors.lastname: Yoon
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Chang Min
authors.identifier: http://orcid.org/0000-0002-2881-4669
authors.initials: CM
authors.lastname: Choi
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Yeon Mok
authors.identifier: http://orcid.org/0000-0003-0116-4683
authors.initials: YM
authors.lastname: Oh
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Sang Do
authors.identifier: http://orcid.org/0000-0001-8189-4509
authors.initials: SD
authors.lastname: Lee
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Chae Man
authors.identifier: http://orcid.org/0000-0001-5400-6588
authors.initials: CM
authors.lastname: Lim
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Woo Sung
authors.identifier: http://orcid.org/0000-0002-1254-1264
authors.initials: WS
authors.lastname: Kim
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
authors.forename: Younsuck
authors.identifier: http://orcid.org/0000-0001-5066-2027
authors.initials: Y
authors.lastname: Koh
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. jsdoc1186@hanmail.net.
authors.forename: Jae Seung
authors.identifier: http://orcid.org/0000-0003-4130-1486
authors.initials: JS
authors.lastname: Lee
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D003131:Combined Modality Therapy; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications; D053208:Kaplan-Meier Estimate; D008175:Lung Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D016016:Proportional Hazards Models; D011651:Pulmonary Artery / Q000473:pathology; D011655:Pulmonary Embolism / Q000175:diagnosis; D056910:Republic of Korea; D012189:Retrospective Studies; D012509:Sarcoma / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D012737:Sex Factors; D016896:Treatment Outcome
keywords: Diagnosis; Pulmonary Artery Sarcoma; Pulmonary Hypertension; Survival; Treatment