Pulmonary artery sarcoma: Case report and review of the literature

Moguillansky, Natalia; Verma, Nupur; Shah, Prithvi; Knapik, Jacquelyn A.; Mohammed, Tan-Lucien H.

doi:10.1016/j.rmcr.2019.100857

Cited by 13 publications

(39 citation statements)

References 16 publications

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“…Immunohistochemically, vimentin, smooth muscle actin (SMA), desmin, actin, and an endothelial cell marker such as CD31 might be expressed . The tumour also express MDM2 . In our case, the morphological and immunohistochemical stain was compatible with the pattern of a sarcoma.…”

Section: Discussionsupporting

confidence: 58%

“…It was first described by Mandelstamm in 1923 . Since then, around 400 cases have been reported . The reported mean age is 50 years .…”

Section: Discussionmentioning

confidence: 99%

“…The radiological features of pulmonary artery sarcoma are filling defect in the pulmonary artery lumen, extraluminal tumour invasion, and a nodular appearance. However, despite specific features, published studies have shown that more than half of the patients with pulmonary artery sarcoma are initially misdiagnosed as pulmonary thromboembolism . Several studies have been conducted in an attempt to distinguish between the two entities.…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary artery sarcomas are characterized by tumour deposits. Thus, the CT features are characteristic with expansive growth and a bulging appearance against the direction of blood flow, a lobulated structure at the proximal part of the tumours, a grape‐like appearance at the distal end of the tumours, and heterogeneous enhancement . Pulmonary artery sarcoma can extend until the bifurcation of the pulmonary arteries, the main pulmonary artery, pulmonary valve, and right ventricular outflow tract .…”

Section: Discussionmentioning

confidence: 99%

“…The T2 signal is higher in a pulmonary artery sarcoma than in thromboembolism. However, patients with pulmonary artery sarcoma usually have dyspnoea and are barely able to hold their breath throughout the MRI procedure .…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary artery intimal sarcoma: a case report and literature review

Chang

Lin

Pan

et al. 2020

Respirology Case Reports

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Pulmonary artery intimal sarcoma is a rare disorder arising from the intima of the pulmonary artery. Histopathology reveals that it is a tumour cell of mesenchymal origin. The signs and symptoms include chronic shortness of breath and other features of right ventricular failure, which mimic chronic pulmonary thromboembolism. The definitive diagnosis can rarely be made based on the symptoms and signs alone, and other investigations including echocardiography, computed tomography, magnetic resonance imaging (MRI), and positron emission tomography (PET) are often required. The gold standard for diagnosis is tissue biopsy. The mainstay for treatment is surgery, and complete surgical resection with endarterectomy provides survival benefit. According to recent evidences, however, multimodal treatment provides better survival outcomes than monotherapy such as surgery alone. Despite the newer upcoming treatment strategies, patients with pulmonary intimal sarcoma continue to have a poor prognosis. We present a case of pulmonary artery intimal sarcoma and review the literature associated with the disease.

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Section: Discussionsupporting

confidence: 58%

“…It was first described by Mandelstamm in 1923 . Since then, around 400 cases have been reported . The reported mean age is 50 years .…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pulmonary artery intimal sarcoma: a case report and literature review

Chang

Lin

Pan

et al. 2020

Respirology Case Reports

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Primary pulmonary artery sarcoma versus pulmonary thromboembolism: a multimodal imaging comparison

Kronzer

Robinson

Collins

et al. 2021

J Thromb Thrombolysis

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Primary pulmonary artery sarcoma (PPAS) is a rare malignancy that is commonly mistaken for pulmonary embolism due to similarities in clinical presentation and radiographic findings. Distinct radiographic findings to help differentiate between the two diseases are highlighted in the case presented. (1) Several nuances in various imaging modalities have been identified to help distinguish pulmonary artery sarcoma from pulmonary thromboembolic disease. (2) The wall eclipsing sign is considered pathognomonic for pulmonary artery sarcoma. (3) Positron emission tomography/computed tomography may help reduce time between diagnosis and treatment, which may ultimately prolong survival. (4) Providers should be well versed on the subtle differences on imaging to prevent future delays in diagnosis and treatment.

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