Commentary: Are we always ready for a challenging surprise?

Central MessagePulmonary artery sarcoma is a rare, but complex pathology requiring multidisciplinary preoperative and postoperative planning, including challenging intraoperative techniques for successful outcomes.See Article page 317. Pulmonary artery sarcoma is a rare, but complex pathology requiring multidisciplinary preoperative and postoperative planning, including challenging intraoperative techniques for successful outcomes. See Article page 317. Pupovac and colleagues1Pupovac S. Hemli J.M. Sarmiento I.C. Lazzaro R.S. Scheinerman S.J. Brinster D.R. The imitation game: pulmonary artery sarcoma masquerading as an acute pulmonary embolism.J Thorac Cardiovasc Surg Tech. 2021; 10: 317-319Scopus (1) Google Scholar present an interesting case report of a rarely seen entity, a pulmonary artery sarcoma (PAS). The images shared demonstrate impressive surgical reconstruction with multiple levels of pulmonary artery repair, including the right ventricle outflow tract with an excellent surgical outcome. Unfortunately, the prognosis of patients with PAS usually is poor. The typical cause of death is right-sided heart failure as a result of the right ventricular outflow obstruction. The clinical presentation of PAS and the radiologic findings are often difficult to interpret, as they can be similar to pulmonary thromboembolism, as in the current case. PAS often presents with symptoms of thromboembolic disease, heart failure, and pulmonary hypertension. Early diagnosis is crucial to perform a curative complete resection. Yeung and colleagues2Yeung D.F. Johnston A. Simmons C. Goddard K. Dewar L.R. Yee J. et al.Multimodality imaging of a pulmonary artery sarcoma.Echocardiography. 2018; 35: 123-125Crossref PubMed Scopus (5) Google Scholar compared different multimodality imaging, including computed tomography (CT), magnetic resonance imaging, and positron emission tomography, to better characterize PAS. Imaging guides the decision and planning for surgical resection and postoperative therapy. Attina and colleagues3Attina D. Niro F. Tchouante P. Mineo G. Russo V. Palazzini M. et al.Pulmonary artery intimal sarcoma. Problems in the differential diagnosis.Radiol Med. 2013; 118: 1259-1268Crossref PubMed Scopus (31) Google Scholar showed that CT characteristics more suggestive of PAS include inhomogeneous high or low attenuation; soft-tissue density with filling defects occupying the entire lumen of the pulmonary trunk along with an increase in diameter of the involved vessel; and patchy and delayed contrast enhancement with CT angiography. Once suspicion is raised, positron emission tomography–CT and magnetic resonance imaging are more helpful to differentiate between sarcoma and pulmonary embolism. Curative surgical resection of the tumor is not always possible due to advanced disease. Debulking of the obstructive tumor may be chosen to restore hemodynamics, but incomplete resection can lead to early relapse. The primary goal of surgery in PAS is restore the blood flow, decompress the right ventricle, and optimally achieve R0 resection margins. Curative surgical procedures for treating the PAS include tumor endarterectomy, graft reconstruction of the pulmonary artery, and pneumonectomy. In addition, as demonstrated in this report, radical resection of the entire pulmonic root, incorporating pulmonic valve replacement and extensive reconstruction of the right ventricular outflow tract, including combination with multimodality treatment, leads to better survival. Blackmon and colleagues4Blackmon S.H. Rice D.C. Correa A.M. Mehran R. Putnam J.B. Smythe W.R. et al.Management of primary pulmonary artery sarcomas.Ann Thorac Surg. 2009; 87: 977-984Abstract Full Text Full Text PDF PubMed Scopus (145) Google Scholar found that patients who undergo an attempt at curative resection have longer overall survival compared with those who do not, with median overall survival of 36.5 versus 11 months. Also, the median survival of those who had received multimodality treatment was found to be superior to those who only had single-modality therapy, with median survival of 24.7 versus 8.0 months. High-volume centers of excellence report similar outcomes, as Wong and colleagues5Wong H.H. Gounaris I. McCormack A. Berman M. Davidson D. Horan G. et al.Presentation and management of pulmonary artery sarcoma.Clin Sarcoma Res. 2015; 5: 3Crossref PubMed Google Scholar had median survival of 17 months, with no difference between patients who also underwent pulmonary endarterectomy. Due to the rarity of PAS, so far only small, retrospective case series have been published over the years. This challenging and successful case report with repeated surgical intervention demonstrates how a multidisciplinary approach can lead to improved results. The imitation game: Pulmonary artery sarcoma masquerading as an acute pulmonary embolismJTCVS TechniquesVol. 10PreviewPrimary sarcoma of the pulmonary artery is rare (0.001%-0.03% of the population) and has been typically associated with a dismal prognosis; after surgical resection and adjuvant therapy, median and 5-year survival have been reported as 20 months and 22%, respectively.1 Treatment often tends to be palliative and symptom-directed, aiming to restore pulmonary blood flow to address any ventilation-perfusion mismatch and to reduce right-sided heart strain.1,2 Herein, we discuss an interesting case of a pulmonary artery sarcoma (PAS) that was initially mistaken for a submassive pulmonary embolism but which was nevertheless managed successfully with better-than-expected oncologic outcomes (as seen in Video 1).