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

Gan, Hui-Li; Zhang, Jianqun; Huang, Xiaoyong; Yu, Wei

doi:10.1371/journal.pone.0083200

Cited by 71 publications

(72 citation statements)

References 24 publications

(23 reference statements)

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“…Chest X-ray examinations of patients with PAS are often nonspecific, and may reveal a hilar mass, protruding pulmonary artery segment, unilateral pulmonary artery, proximal branch expansion, lung nodules, right ventricular enlargement and sparse peripheral vasculature (8,19). CTPA and pulmonary magnetic resonance imaging (MRI) present advantages for the diagnosis of PAS (7,18,20). CTPA may clearly reveal the involvement of lesions in the pulmonary artery, right ventricular outflow tract and pulmonary valve (17).…”

Section: Discussionmentioning

confidence: 99%

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Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

et al. 2016

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Abstract. Pulmonary artery sarcoma (PAS) is an extremely rare and highly malignant tumor that originates in the pulmonary artery. The majority of reported cases of PAS are confirmed by pathological examination subsequent to surgery or by autopsy. The present study reports the clinicopathological characteristics and immunohistochemical phenotypes of three cases of PAS, and aims to facilitate the identification of this lethal disease. In the present study, the data from clinical, histopathological and immunohistochemical examinations of three patients with PAS, whose diagnoses were confirmed by surgical biopsy conducted at the Beijing Anzhen Hospital (Beijing, China) between 2008 and 2012, were retrospectively analyzed. The patients (two women and one man; average age, 41.3 years old) presented with dyspnea on exertion. In addition, two of the patients experienced chest tightness, and one patient experienced intermittent syncope. Computed tomography pulmonary angiography revealed that two of the patients possessed a filling defect in the main, left and right pulmonary arteries, and one patient possessed a filling defect in the right upper pulmonary artery. Macroscopically, the PAS appeared as a mucoid intraluminal or nodular sessile mass spreading along the pulmonary artery. Microscopically, the tumor consisted of spindle cells with fascicular and storiform patterns, and was accompanied by necrosis and stromal myxoid changes. Immunohistochemically, vimentin, desmin and cluster of differentiation 34 were highly expressed in the patient that was diagnosed with intimal sarcoma, while vimentin and α-smooth muscle actin were highly expressed in the other two patients, who were diagnosed with leiomyosarcoma. PAS is often misdiagnosed due to nonspecific clinical manifestations and radiological features. Therefore, the diagnosis of PAS should be based on typical morphological features and immunohistochemical analysis of the tumor tissue. IntroductionPulmonary artery sarcoma (PAS) is an extremely rare and highly malignant tumor that originates in the pulmonary artery (1). It has an incidence rate of 0.001-0.030% (2). Since 1923, when the first case of PAS was reported by Mandelstamm (3), ≤300 cases have been reported to date worldwide (4-7). The etiology of PAS is unknown, and this disease has a poor prognosis (5). Early diagnosis followed by radical surgical resection constitutes the only chance of survival for patients with PAS (5), and patients have an average survival time of ~1.5 months without surgical treatment (8). However, due to the rare and nonspecific clinical manifestations and imaging findings, PAS is frequently misdiagnosed as various pulmonary thromboembolic diseases, including pulmonary thromboembolism (PTE) or chronic thromboembolic pulmonary hypertension (CTEPH), and the majority of reported PAS cases are confirmed by pathological examination subsequent to surgery or by autopsy (2,9,10). The present study reports three cases of PAS that were initially misdiagnosed as PTE or CTEPH, and were later identifi...

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Section: Discussionmentioning

confidence: 99%

“…It has an incidence rate of 0.001-0.030% (2). Since 1923, when the first case of PAS was reported by Mandelstamm (3), ≤300 cases have been reported to date worldwide (4)(5)(6)(7). The etiology of PAS is unknown, and this disease has a poor prognosis (5).…”

Section: Introductionmentioning

confidence: 99%

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

et al. 2016

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“…The majority of patients have demonstrated insidious onset, predominantly involving progressive dyspnea, chest pain, coughing, hemoptysis, syncopes, fever, fatigue and weight loss (4,6,8,15,16). X-rays of these patients typically show a prominent hilar shadow, sparse texture of the peripheral vasculature, lung nodules and enlargement of the heart.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, previous studies have recommended that the CT data be used together with positron-emission tomography (PET)-CT results for the diagnosis of PAS (4,(7)(8)(9). The performance of 18 F-fludeoxyglucose ( 18 FDG) PET-CT in patients with PTE or CTEPH showed no increase in radiotracer intake, whereas in patients with PAS, 18 FDG PET-CT showed an increase in radiotracer uptake, which may help to differentiate between the two diseases.…”

Section: Discussionmentioning

confidence: 99%

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Clinical Features of Pulmonary Artery Sarcoma-a Report of Three Cases

Zhu

2016

Chest

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Pulmonary artery sarcoma: An important mimic of pulmonary embolism—Case reports and literature review

Tuft

Maheepala

Raguparan

et al. 2022

Respirology Case Reports

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Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low d ‐dimer, troponin T or NT‐proBNP, as well as characteristic findings on CT pulmonary angiography such as the ‘wall eclipsing sign’ and an non‐dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2‐year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.

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

Cited by 71 publications

References 24 publications

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

Clinicopathological and immunohistochemical features of pulmonary artery sarcoma: A report of three cases and review of the literature

Clinical Features of Pulmonary Artery Sarcoma-a Report of Three Cases

Pulmonary artery sarcoma: An important mimic of pulmonary embolism—Case reports and literature review

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