Surgical treatment of pulmonary artery sarcoma: a report of 17 cases

Abstract

Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis is often delayed, and optimal treatment remains unclear. The aim of this study is to report our experience in the surgical management of this disease. Between 2000 and 2018, 17 patients underwent operations for PAS at our center. The medical records were retrospectively reviewed to evaluate the clinical characteristics, operative findings, the postoperative outcomes, and the long-term results. The mean age at operation was 46.0 ± 12.4 years (range, 26–79 years), and eight (47.1%) patients were male. Six patients underwent tumor resection alone, whereas the other 11 patients received pulmonary endarterectomy (PEA). There were two perioperative deaths. Follow-up was completed for all patients with a mean duration of 23.5 ± 17.6 months (1–52 months). For all 17 patients, the median postoperative survival was 36 months, and estimated cumulative survival rates at 1, 2, 3, and 4 years were 60.0%, 51.4%, 42.9%, and 21.4%, respectively. The mean survival was 37.0 months after PEA and 14.6 months after tumor resection only ( p = 0.046). Patients who had no pulmonary hypertension (PH) postoperatively were associated with improved median survival (48 vs. 5 months, p = 0.023). In conclusion, PAS is often mistaken for chronic pulmonary thromboembolism. The prognosis of this very infrequent disease remains poor. Early detection is essential for prompt and best surgical approach, superior to tumor resection alone, and PEA surgery with PH relieved can provide better chance of survival.

Missed pulmonary artery sarcoma requiring radical excision and pneumonectomy

“…1 Regardless, once sarcoma is suspected, early and complete surgical resection has been consistently demonstrated as the most important curative technique in PAS management. [2][3][4] Despite this, bilateral PAS has been conventionally managed with palliative pulmonary endarterectomy, forgoing complete resection for symptomatic relief. 2,3 With the goal of complete curative excision, we decided intraoperatively to continue with a radical resection and pneumonectomy.…”

“…[2][3][4] Despite this, bilateral PAS has been conventionally managed with palliative pulmonary endarterectomy, forgoing complete resection for symptomatic relief. 2,3 With the goal of complete curative excision, we decided intraoperatively to continue with a radical resection and pneumonectomy. This required extensive planning, including ensuring homograft availability for reconstruction of the pulmonary artery.…”

“… 2 , 3 , 4 Despite this, bilateral PAS has been conventionally managed with palliative pulmonary endarterectomy, forgoing complete resection for symptomatic relief. 2 , 3 …”

Treatment outcomes in primary pulmonary angiosarcoma: an analysis based on the SEER database

“…Surgical resection, chemotherapy and radiotherapy are the main methods for the treatment of PPA reported in the paper, but they are all derived from case reports and follow-up summaries of a few cases. In addition, there are no treatment guidelines and expert consensus for reference, and the reported clinical outcomes are mixed [6][7][8][9][10][11][12][13] . We used the SEER database to analyze data from previous PPA cases to explore real-world outcomes for this extremely rare tumor of the lung.…”

Complete resection of a pulmonary artery sarcoma involving the pulmonary valve and right ventricle outflow tract: a case report

“…Since the first report on pulmonary artery sarcoma (PAS) by Mandelstamm in 1923, <500 cases of PAS have been published so far. 1 , 2 The mainstay of treatment for PAS is surgery; however, less than half of patients with PAS are suitable for complete resection intra-operatively. 2 As previously published, 1 , 3 , 4 the median survival after PAS is 17–36 months even after surgery, since most patients develop local tumour recurrence or distant metastases.…”