K. YIN1, Y. Lin1, Z. Zhang1, C. Wang1 1Zhongshan Hospital Fudan University,Department Of Cardiac Surgery,Shanghai, SHANGHAI, China
Introduction: Pulmonary artery sarcoma (PAS) is a rare and poorly understood malignancy that arises from the intimal layer of pulmonary artery. There is lack of consensus on the diagnosis and treatment because of its rarity. We reviewed our experience of surgically management of PAS patients during the past 11 years.
Methods: From 2005 to 2016, 12 patients (age 51±14 years, 5 males) were ultimately diagnosed as PAS and underwent surgical treatment at our center. Chest pain (10/12) and asthenia (7/12) were two most common symptoms with all the patients presenting different extent of pulmonary artery hypertension. Six patients were initially misdiagnosed as pulmonary embolism. Ten patients underwent pulmonary endarterectomy (PEA) with cardiopulomonary bypass (83 ± 39 mins), among which three patients required pneumonectomy as the tumors invaded distal pulmonary arteries. The rest two underwent exploratory thoracotomy (including one emergency case) without being able to resect the tumors.
Results: There was one in-hospital mortality who underwent emergent thoracotomy for prominent internal bleeding and died at postoperative day 15. Follow-up was 100% complete. One patients underwent reoperation 52 months after the first operation but died four months later. The median overall survival was 18.5 months. All late death (7/12) were related to cancer recurrence or metastasis. PAS with myofibroblastic differentiation (5/12) and postoperative adjuvant treatment (isolated chemotherapy, isolated radiotherapy, or combined chemotherapy and radiotherapy, 7/12) carried better overall survival but neither of which reached statistical significance (p = 0.094 and 0.164, respectively).
Conclusion: PAS has a very poor prognosis. Surgical treatment, PEA combined with pneumonectomy, provides the only chance of long-term survival. Particular histological type and postoperative adjuvant treatment may be associated with better survival but still require further studies.