ADULT – Original Submission| Volume 35, ISSUE 1, P53-64, March 2023

Surgical Management of Primary Pulmonary Artery Sarcoma

Published:November 03, 2021DOI:
      Primary pulmonary artery sarcoma is a rare cardiac tumor with a dismal prognosis without surgical therapy. It is often confused with the more common chronic pulmonary emboli which may delay the appropriate diagnosis or lead to suboptimal surgery. The objective of this study was to evaluate the short and long-term survival and local recurrence rate of pulmonary artery sarcoma cases operated on at our institution using an anatomic resection approach for the pulmonary trunk and main pulmonary arteries rather than endarterectomy. We searched our prospectively collected cardiac tumor database for cases of primary pulmonary sarcoma operated at our institution between June 2000 and September 2018 and followed until January 3, 2021. We used an anatomic resection and replacement technique for involved pulmonary root and main pulmonary arteries with endarterectomy used only for disease distal to the first arterial branch when lung preservation was possible. The primary endpoints for our study were survival from the time of initial diagnosis and survival from the time of our surgery. Secondary endpoints were operative 30-day mortality and incidence of local recurrence or metastatic disease. We identified 20 consecutive cases of surgical resection of primary pulmonary sarcoma. The median age at surgery was 52.5 years (IQR 43.5–60.5). Complete pulmonary root resection and reconstruction using a pulmonary homograft were needed in 16/20 (80%) of cases. All resections employed cardiopulmonary bypass with cardioplegic arrest. A pneumonectomy was needed in 7/20 (35%) of patients. A negative margin (R0) resection was achieved in 9 patients (45%) and margins were microscopically positive (R1) on final pathology in 9 patients (45%). Two patients (10%) had gross tumor (R2) at the resection margin. Operative mortality was 2/20 (10%). Median survival was 2.8 years from diagnosis (95% CI 1.3–8.8) and 2.7 years from surgery by our team (95% CI 0.8–5.9). Survival from first initial diagnosis at 1, 3, 5, and 10 years was 85.0%, 49.1%, 49.1%, and 16.4%. Survival from our surgery by our team at 1, 3, 5, and 10 years was 70%, 48.8%, 41.8%, and 8.4%. Surgical resection of primary pulmonary artery sarcoma with an approach utilizing an anatomic resection of the pulmonary root and main pulmonary arteries when involved and pneumonectomy or endarterectomy when there is disease distal to the first branch artery can be done with a reasonable operative risk and long-term survival when compared to the natural history of the disease.

      Graphical Abstract



      IQR (interquartile range), CT (computed tomography), CMR (cardiac magnetic resonance imaging), CPB (cardiopulmonary bypass), R0 (pathologically negative surgical margin), R1 (microscopically positive surgical margin), R2 (grossly positive surgical margin), CI (Confidence interval)
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      Linked Article

      • Commentary: Improving Surgical Outcomes for Pulmonary Artery Sarcoma: The Challenge Remains
        Seminars in Thoracic and Cardiovascular SurgeryVol. 35Issue 1
        • Preview
          Primary pulmonary artery sarcoma (PAS) is a rare thoracic tumor with a dismal prognosis.1 Since the report of Mandelstramm in 1923,2 there have been very few case series with more than 10 patients.3,4 Because of this, the clinical features, diagnostic modalities, surgical approaches, and chemotherapy/radiotherapy remain controversial.5,6
        • Full-Text
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      • Commentary: “It's a Heartache 2.0″
        Seminars in Thoracic and Cardiovascular SurgeryVol. 35Issue 1
        • Preview
          In a recent editorial “It's a Heartache,”1 the Houston Methodist and MD Anderson Cancer Institute's surgical experience with 122 primary cardiac sarcomas (PCS) patients2 was likened to the verse “It's Nothing But a Heartache” from the 1977 Bonnie Tyler release It's A Heartache.1 Consequently, this review of the Primary Pulmonary Artery Sarcoma (PPAS) resection cohort from the same institution is entitled “It's A Heartache 2.0″ as it summarizes a sub-group from the initial report.
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