Pulmonary artery intimal sarcoma. Problems in the differential diagnosis

Attinà, Domenico; Niro, Fabio; Tchouanté, Patrick; Mineo, Giangaspare; Russo, Vincenzo; Palazzini, Massimiliano; Galiè, Nazzareno; Fanti, Stefano; Lovato, Luigi; Zompatori, Maurizio

doi:10.1007/s11547-013-0943-x

Cited by 41 publications

(47 citation statements)

References 14 publications

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“…Echocardiography of the large pulmonary artery and right ventricular outflow tract may reveal projections with irregular surfaces as spot echoes and hyperechoic regions. However, distinguishing PAS from pulmonary thromboembolic diseases such as PTE or CTEPH is challenging (18). 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).…”

Section: Discussionmentioning

confidence: 99%

“…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%

Section: Discussionmentioning

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%

“…Clinically, PAS can easily be misdiagnosed as a pulmonary thromboembolic disease, such as pulmonary thromboembolism (PTE) or chronic thromboembolic pulmonary hypertension (CTEPH) (5-7). In such instances, patients lose the opportunity for early diagnosis and treatment (8,9). In the present study, the clinical data of three patients with PAS, as confirmed by surgery at the Beijing Anzhen Hospital (Beijing, China), are reported and analyzed, in order to raise awareness and improve the diagnosis of PAS.…”

Section: Introductionmentioning

confidence: 99%

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

Zhu

2016

Chest

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An intimal sarcoma of pulmonary artery mimicking pulmonary embolism: a case report and literature review

Liu

Hou

Wang

et al. 2017

Respirology Case Reports

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Pulmonary artery intimal sarcoma is a highly aggressive disease, and is most often misdiagnosed as pulmonary thromboembolism (PTE) due to the similar clinical symptoms and its rarity, which leads to the use of inappropriate treatments such as prolonged anticoagulant therapy. We reported a case of pulmonary artery intimal sarcoma in a patient who was misdiagnosed as having PTE. Pathology after surgery confirmed malignant disease. We concluded that when a patient presents with mild clinical manifestations yet with strong imaging manifestations, pulmonary artery malignancy should be suspected.

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Pulmonary artery intimal sarcoma. Problems in the differential diagnosis

Cited by 41 publications

References 14 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

An intimal sarcoma of pulmonary artery mimicking pulmonary embolism: a case report and literature review

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