There are limited data regarding the surgical management of primary pulmonary artery sarcomas (PPAS) because of their rarity and complicated diagnostic history. The objective of this study was to analyze our institution’s long-term surgical management outcomes for PPAS in the absence of a care pathway. From May 1997 to June 2013, 8 patients (mean age 60.6 ± 11.8 years; range, 40-73 years; 5 women and 3 men) underwent surgical intervention for PPAS at our institution. The most common computed tomography finding was a luminal filling defect obstructing the pulmonary artery (PA), without evidence of extraluminal extension. Three patients underwent debulking/pulmonary endarterectomy alone and 5 patients underwent a more radical resection with PA patch angioplasty, PA resection and reconstruction, pulmonary valve replacement, and unilateral pneumonectomy. The mean postoperative survival in this series was 3.8 ± 3.6 years (range, 1-11.9 years), with 2 radical surgical resection patients alive at 4.9 and 11.9 years, respectively. For those patients with incomplete resection, 3-dimensional (3D) models were created to demonstrate the advantage of a preoperative guide for a more complete resection and what it would entail. Six patients had local recurrences with mean disease-free interval of 14 ± 10.9 months (range, 2 months-2.5 years), and 2 patients with re-resections had an overall postoperative survival of 2.8 and 11.9 years, respectively. In our small cohort of PPAS, patients treated with radical surgical resection had better survival. The small number of PPAS cases in this series makes proving this association unlikely but warrants consideration.
|Original language||English (US)|
|Number of pages||7|
|Journal||Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery|
|State||Published - Jan 2021|
Bibliographical noteFunding Information:
We thank Carl G. Clingman for creating the medical illustration images of surgical resections techniques of PPAS; Dr Jane M. Matsumoto, Dr Jonathan M. Morris, Amy Alexander, Brenda Thompson, and the Mayo Clinic Medical 3D Printing Lab for creation of the 3D printed anatomic models of PPAS; Karlyn E. Pierson, Bettie J. Lechtenberg, and the thoracic surgery research team for IRB protocol submission and patient evaluation. The author(s) received no financial support for the research, authorship, and/or publication of this article.
© The Author(s) 2020.
- 3D printing
- 3D reconstruction
- pulmonary artery endarterectomy
- pulmonary artery sarcoma
- surgical resection
PubMed: MeSH publication types
- Journal Article