Başar Veysel, Ermerak N Onur, Olgun Yıldızeli Şehnaz, Bozkurtlar Emine, Ercelep Özlem, Mutlu Bülent, Kocakaya Derya, Bekiroğlu G Nural, Taş Serpil, Yanartaş Mehmed, Sunar Hasan, Ak Koray, Küçükoğlu Serdar, Yıldızeli Bedrettin
Department of Cardiovascular Surgery, University of Health Sciences, Koşuyolu High Specialization Education and Research Hospital, Istanbul, Türkiye.
Department of Thoracic Surgery, Marmara University Faculty of Medicine, Istanbul, Türkiye.
Turk Gogus Kalp Damar Cerrahisi Derg. 2023 Jul 27;31(3):388-397. doi: 10.5606/tgkdc.dergisi.2023.23906. eCollection 2023 Jul.
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.
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.
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 to 191 dyn/s/cm (p<0.004). All patients received chemotherapy following surgery. Median followup 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.
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.
在本研究中，我们旨在描述接受肺动脉内膜剥脱术的原发性肺动脉肉瘤患者的治疗经验，并根据这种恶性疾病的组织学亚型评估临床特征、治疗方法、结局和生存率。
回顾性分析2011年3月至2022年5月期间共13例接受肺动脉内膜剥脱术并被诊断为肺动脉肉瘤的患者（7例男性，6例女性；平均年龄：52.6±13.0岁；范围30至69岁）。所有患者均经组织病理学确诊。记录包括人口统计学、临床特征、术中和术后并发症、住院时间、发病率、死亡率以及短期和长期结局等数据。手术死亡率定义为在医院内或手术后30天内死亡。
1例患者因大量咯血死亡。2例患者因急性呼吸窘迫出现并发症。肺血管阻力从508 dyn/s/cm显著改善至191 dyn/s/cm（p<0.004）。所有患者术后均接受化疗。中位随访时间为14个月。整个队列的中位生存期为18个月。1年和3年生存率分别为60.6%和30.3%。平滑肌肉瘤（n=6）的中位生存期为7个月，而内膜肉瘤为44个月（p=0.004）。内膜肉瘤的3年生存率为66.7%，平滑肌肉瘤为0%。
肺动脉肉瘤可能类似慢性血栓栓塞性肺动脉高压。疑似肺动脉肉瘤诊断的患者应转诊至有多学科团队的专业肺动脉内膜剥脱术中心进行手术。肺动脉内膜剥脱术具有诊断和治疗价值，可能改善生存率和生活质量。与平滑肌肉瘤患者相比，内膜肉瘤患者的生存期更长。
