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      Clinical Characteristics and Treatment Outcomes of Primary Pulmonary Artery Sarcoma in Korea

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          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.

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          Most cited references 36

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          Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases.

          To investigate the characteristics and pathological features of primary cardiac tumors and to evaluate the diagnostic sensitivity of echocardiography in primary cardiac tumors, all pathologic and echocardiographic records at the Chinese PLA general hospital and its satellite hospitals between January 1st, 1990 and January 1st, 2000 were reviewed to identify patients with a confirmed diagnosis of primary cardiac tumors. A total of 149 patients who had complete echocardiographic records and who were diagnosed with primary cardiac tumors were included in the study. Pathologic and echocardiographic records were reviewed retrospectively to evaluate the presence, location and histologic type of the tumors. The majority (n=118, 79.2%) of cases had been diagnosed with benign tumors. Myxoma was the most common histologic type accounting for 50.0% of total cardiac tumors. Lipoma was the second most common type of benign tumor. Among cases with malignant tumors (n=31, 20.8%), unclassified sarcoma (n=7), angiosarcoma (n=6) and rhabdomyosarcoma (n=6) were the common histologic types of primary malignant tumor. Non-myxomatous benign tumors were more likely to have occurred in the ventricle than myxomas (17/43, 39.5% vs. 7/75, 9.3%; P=0.00). The proportion of pericardium involvement in the malignant tumors (8/31, 25.8%) was significantly higher than that in the myxomas (0/75, 0%; P=0.00) and non-myxomas (2/43, 4.7%; P=0.01). The diagnostic sensitivity of transthoracic and transesophageal echocardiography was 93.3% (139/149) and 96.8% (30/31), respectively. The study, using a relatively large sample, confirms that myxoma was the most common primary cardiac tumor. The locations of tumor involvement varied by types of tumor. Echocardiography may be a useful tool for early diagnosis of primary cardiac tumors.
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            Management of primary pulmonary artery sarcomas.

            The objective of this review is to determine the outcome of patients with sarcomas involving the main pulmonary artery, pulmonic valve, or right ventricular outflow tract. Survival data were analyzed using an aggregate series derived from the published literature in conjunction with a current series. Median survival was 36.5 +/- 20.2 months for patients undergoing an attempt at curative resection compared with 11 +/- 3 months for those undergoing incomplete resection. Median survival was 24.7 +/- 8.5 months for patients undergoing multimodality treatment compared with 8.0 +/- 1.7 months for patients having single-modality therapy. A complete review of diagnosis, evaluation, treatment, and surveillance of primary pulmonary artery sarcomas follows.
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              Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma.

              The study aimed to determine the optimal surgical procedure to treat pulmonary artery sarcomas responsible for pulmonary hypertension. Between 1997 and 2010, 31 patients were treated surgically for pulmonary artery sarcomas. Sixteen patients were male; the mean age was 56 years (range, 26-78 years). Common symptoms were characteristic of acute or chronic pulmonary thromboembolic disease. Also, 21 patients experienced mild to severe pulmonary hypertension, with a mean total peripheral resistance of 473 dyn s cm(-5). Clinical presentation and preoperative work-up confirmed the suspicion of pulmonary artery sarcoma in 18 patients. The required surgical procedures included the following: pulmonary endarterectomy in 25 patients (combined with a right pneumonectomy in five and with a replacement of the main pulmonary artery by a homograft reconstruction in one), pneumonectomy only in five (three right and two left), with the use of cardiopulmonary bypass in three cases. In one patient, the right pulmonary artery only was replaced on cardiopulmonary bypass. Final pathology showed 26 high-grade and five intermediate-grade sarcomas. The 30-day mortality was 13% (four patients). Repeat pulmonary resection was required in two patients due to recurrent disease. Moreover, 18 patients received adjuvant therapy. Mean follow-up was 19 months (range, 1-99 months); of the 11 patients alive at follow-up, four were noted to have recurrent disease. The 1-, 3- and 5- year survival was 63, 29 and 22%, respectively. The prognosis of this very infrequent disease remains poor. Bilateral pulmonary endarterectomy may yield significant survival rates because it provides completeness of resection without sacrificing the pulmonary vascular bed.
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                Author and article information

                Journal
                J Korean Med Sci
                J. Korean Med. Sci
                JKMS
                Journal of Korean Medical Science
                The Korean Academy of Medical Sciences
                1011-8934
                1598-6357
                November 2016
                19 August 2016
                : 31
                : 11
                : 1755-1760
                Affiliations
                [1 ]Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
                [2 ]Department of Internal Medicine, Kyungpook National University Hospital, Kyungpook National University School of Medicine, Daegu, Korea.
                Author notes
                Address for Correspondence: Jae Seung Lee, MD. Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea. jsdoc1186@ 123456hanmail.net
                Article
                10.3346/jkms.2016.31.11.1755
                5056207
                27709853
                © 2016 The Korean Academy of Medical Sciences.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Original Article
                Respiratory Diseases

                Medicine

                diagnosis, pulmonary artery sarcoma, pulmonary hypertension, survival, treatment

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