ISSN : 1301-5680
e-ISSN : 2149-8156
Turkish Journal of Thoracic and Cardiovascular Surgery     
Results of surgical treatment of pulmonary artery sarcomas: Does histology affect survival?
Veysel Başar1, N. Onur Ermerak2, Şehnaz Olgun Yıldızeli3, Emine Bozkurtlar4, Özlem Ercelep5, Bülent Mutlu6, Derya Kocakaya3, G. Nural Bekiroğlu7, Serpil Taş1, Mehmed Yanartaş1, Hasan Sunar1, Koray Ak8, Serdar Küçükoğlu9, Bedrettin Yıldızeli2
1Department of Cardiovascular Surgery, University of Health Sciences, Koşuyolu High Specialization Education and Research Hospital, Istanbul, Türkiye
2Department of Thoracic Surgery, Marmara University Faculty of Medicine, Istanbul, Türkiye
3Department of Pulmonology and Intensive Care, Marmara University Faculty of Medicine, Istanbul, Türkiye
4Department of Pathology, Marmara University Faculty of Medicine, Istanbul, Türkiye
5Department of Medical Oncology, Marmara University Faculty of Medicine, Istanbul, Türkiye
6Department of Cardiology, Istanbul University Institute of Cardiology, Istanbul, Türkiye
7Department of Biostatistics, Marmara University Faculty of Medicine, Istanbul, Türkiye
8Department of Cardiovascular Surgery, Marmara University Faculty of Medicine, Istanbul, Türkiye
9Department of Cardiology, Istanbul University Institute of Cardiology, Istanbul, Türkiye
DOI : 10.5606/tgkdc.dergisi.2023.23906
Background: In this study, we aimed to describe our experience with primary pulmonary artery sarcoma in patients who underwent pulmonary endarterectomy and to evaluate clinical features, treatment, outcomes, and survival rates according to the histological subtypes of this malignant disease.

Methods: Between March 2011 and May 2022, a total of 13 patients (7 males, 6 females; mean age: 52.6±13.0 years; range, 30 to 69 years) who underwent pulmonary endarterectomy and diagnosed with a pulmonary artery sarcoma were retrospectively analyzed. The diagnosis was confirmed histopathologically in all patients. Data including demographics, clinical characteristics, intra- and postoperative complications, length of hospital stay, morbidity, mortality, and short-term and long-term outcomes were recorded. Operative mortality was defined as death in the hospital or within 30 days of surgery.

Results: Mortality was observed in one patient due to massive hemoptysis. Morbidity developed in two patients due to acute respiratory distress. Pulmonary vascular resistance improved significantly from 508 dyn/s/cm-5 to 191 dyn/s/cm-5 (p<0.004). All patients received chemotherapy following surgery. Median follow-up was 14 months. Median survival for the entire series was 18 months. One-year and three-year survival rates were 60.6% and 30.3%, respectively. Median survival for leiomyosarcomas (n=6) was seven months, while it was 44 months for intimal sarcomas (p=0.004). Three-year survival was 66.7% for intimal sarcomas and 0% for leiomyosarcomas.

Conclusion: Pulmonary artery sarcoma may mimic chronic thromboembolic pulmonary hypertension. Patients with a suspected diagnosis of pulmonary artery sarcoma should be referred to expert pulmonary endarterectomy centers for surgery where a multidisciplinary team is available. Pulmonary endarterectomy has both diagnostic and therapeutic value and may improve survival and quality of life. Patients with intimal sarcoma have longer survival compared to those with leiomyosarcoma.

Keywords : Malignancy, pulmonary artery sarcoma, pulmonary endarterectomy, sarcoma
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