Surgical treatment of pulmonary artery sarcoma

Gan, Hui-Li; Zhang, Jianqun; Zhou, Qiwen; Xiao, Wei; Gao, Yuan-ming; Liu, Shuang; Wang, Pi-Shan

doi:10.1016/j.jtcvs.2011.03.013

Cited by 20 publications

(24 citation statements)

References 16 publications

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“…The prognosis of PAS is therefore extremely poor. The natural course of the disease is determined by local tumor growth as well as superimposed thrombosis and metastasized tumor embolus, leading to progressive obstruction of the pulmonary vessels [10], [11]. The median survival of time of untreated patients after diagnosis is 1.5 months (range 1.5–5.5 months) [12], [13].…”

Section: Discussionmentioning

confidence: 99%

The Wall Eclipsing Sign on Pulmonary Artery Computed Tomography Angiography Is Pathognomonic for Pulmonary Artery Sarcoma

et al. 2013

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BackgroundThe objective of this study was to evaluate the imaging characteristics of pulmonary artery sarcoma (PAS) on pulmonary artery computed tomography angiography (PACTA) that can be used to differentiate between PAS and pulmonary thromboembolic diseases, including chronic thromboembolic pulmonary hypertension (CTEPH) and acute pulmonary embolism (APE).MethodsThe clinical data and imaging characteristics of 12 patients with PAS, 156 patients with CTEPH, and 426 patients with APE who were treated at Beijing Anzhen Hospital from January 2007 to August 2013 were retrospectively analyzed. All patients underwent PACTA before treatment, and the diagnoses of PAS and CTEPH were all confirmed by surgical biopsy.ResultsAll 12 PAS patients were initially misdiagnosed and received inappropriate thrombolytic and/or anticoagulant therapy before they were referred for surgical intervention. The mean time from PACTA to surgical intervention was 5.5±3.7 months (range 2–11 months). On PACTA, the PAS lesion always eclipsed the wall of the pulmonary artery before infiltrating outside the pulmonary artery, which was termed the wall eclipsing sign. This sign was observed in all PAS patients but was not observed in any CTEPH or APE patients.ConclusionsPAS is a rare neoplasm with a poor prognosis, and is easily misdiagnosed as thromboembolic disease. The wall eclipsing sign on PACTA is pathognomonic for PAS, and patients with this sign should be investigated to confirm the diagnosis and should undergo surgical intervention as soon as possible, rather than receiving thrombolytic or anticoagulant therapy.

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Section: Discussionmentioning

confidence: 99%

The Wall Eclipsing Sign on Pulmonary Artery Computed Tomography Angiography Is Pathognomonic for Pulmonary Artery Sarcoma

et al. 2013

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“…We retrospectively reviewed their clinical records to evaluate the patient characteristics, perioperative findings, and postoperative outcomes. There were 14 articles, which reported at least 6 each, that identified 201 patients diagnosed with PAS, and 158 patients had detailed treatment and follow-up data [7][8][9][10][11][12][13][14][15][16][17][18][19][20].…”

Section: Methodsmentioning

confidence: 99%

“…They found that the 20 patients receiving PEA alone seemed to have a more prolonged survival duration than the 11 patients who underwent pneumonectomy [13]. Gan et al [14] concluded that only PAS resection without distal segmental pulmonary embolectomy might leave the superimposed thrombosis and metastasized PAS emboli, which lead to progressive obstruction in the distal segmental PA [29]. In their study, the median survival time of the five patients without distal embolectomy was 10 months; in contrast, the four patients who underwent distal embolectomy achieved 30-43 months of survival at follow-up.…”

Section: Pneumonectomymentioning

confidence: 99%

“…Before the twenty-first century, pneumonectomy with or without PA replacement was the mainstay surgical procedure [17,20,27,28]. The overall unilateral PAS rate is only 30% (44/145) among the reported cases [7,[9][10][11][12][14][15][16][17][18]. Grazioli et al concluded that pneumonectomy might be a possible radical resection technique for the base of unilateral disease, achieving a median survival 26.8 months longer than the 6.6 months in the PTE group [12].…”

Section: Pneumonectomymentioning

confidence: 99%

“…Blackmon thinks that if PV excision is necessary for R0 resection, then a homograft can be used for PV reconstruction [15]. Gan et al peeled the tumor carefully from the PV and PA without PV reconstruction [14]. Furthermore, Deng et al performed 3 PEA combined with PV replacement (PVR) for six patients with PV involvement [7], and the patients who underwent PVR achieved a longer survival time (29.3 months vs. 7 months).…”

Section: Pulmonary Valve Involvementmentioning

confidence: 99%

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Surgical treatment of primary pulmonary artery sarcoma

Han

Zhen

Liu

et al. 2020

Gen Thorac Cardiovasc Surg

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Objective Primary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS. Methods From September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data. Results All of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7–28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve. Conclusions PAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.

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