Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma†

Mussot, Sacha; Ghigna, Maria-Rosa; Mercier, Olaf; Fabre, Dominique; Fadel, Élie; Cesne, Axel Le; Simonneau, Gérald; Dartevelle, Philippe

doi:10.1093/ejcts/ezs387

Cited by 127 publications

(154 citation statements)

References 24 publications

Supporting

Mentioning

144

Contrasting

Unclassified

Order By: Relevance

“…PAS has been previously reported in patients aged 13-86 years, with a mean age of 52 years old, and a female-to-male ratio of 2:1 (9). The three patients reported in the present study are consistent with the previously reported age and gender characteristics of the disease (15). PAS generally occurs insidiously (15).…”

Section: Discussionsupporting

confidence: 92%

See 1 more Smart Citation

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

et al. 2016

View full text Add to dashboard Cite

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...

show abstract

Section: Discussionsupporting

confidence: 92%

“…The three patients reported in the present study are consistent with the previously reported age and gender characteristics of the disease (15). PAS generally occurs insidiously (15). A tumor that protrudes toward the pulmonary artery lumen may cause the reduction of pulmonary blood volume and pulmonary hypertension (16).…”

Section: Discussionsupporting

confidence: 91%

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

et al. 2016

View full text Add to dashboard Cite

show abstract

“…It was first described in 1923 by Mandelstamm 5 in a study arising from an autopsy, and, since then, a couple of hundred cases of primary PA sarcoma have been described. 6 The prognosis is poor, with survival periods ranging from 6 months to 2 years, and the combination …”

Section: Discussionmentioning

confidence: 99%

“…It has been reported that bilateral pulmonary endarterectomy might yield significant survival rates because it provides completeness of resection without sacrificing the pulmonary vascular bed. 6 These tumors arise from the intima and rarely extend through the adventitia to invade surrounding structures, so resection might result in complete removal. Because the tumor often involves vital cardiac structures such as the pulmonary valve (30% of cases), radical surgical resection can be challenging.…”

Section: A B D Cmentioning

confidence: 99%

Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism

Coşkun¹,

Sinan²,

Calpar³

et al. 2014

Texas Heart Institute Journal

View full text Add to dashboard Cite

We describe the case of a 60yearold woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiag nosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarter ectomy.Early P ulmonary artery (PA) sarcoma, a very rare tumor of the cardiovascular system, is often misdiagnosed as acute or chronic pulmonary thromboembolism because its clinical presentation and radiologic findings resemble those of thromboembolism.1,2 The prognosis is usually poor: the tumor is invasive and often involves vital structures such as the heart, which makes radical resection challenging. 3 We describe the case of a 60-year-old woman with PA sarcoma that was initially misdiagnosed. Case ReportIn February 2013, a 60-year-old woman presented at our emergency department with progressive dyspnea and palpitation. The dyspnea had started after a long bus ride. Her New York Heart Association (NYHA) functional class was II/III. On physical examination, she was positive for jugular venous distention. Auscultation revealed a grade 3/6 systolic ejection murmur in the left parasternal and 2nd intercostal areas. Her hemodynamic state was stable. Her electrocardiogram showed sinus rhythm and a pattern of right ventricular (RV) strain. The patient's cardiothoracic ratio was greater than 50%, and her natriuretic pro-brain peptide level was high. Transthoracic echocardiography (TTE) and computed tomography (CT) of the chest showed RV dilation with impaired function and a mass (2.7 × 1.3-cm) extending from the pulmonary valve into the right and left PAs and into all sub-branches, which suggested a pulmonary embolus (Figs. 1 and 2). Systolic PA pressure (sPAP) derived by Doppler echocardiography was 122 mmHg. Laboratory investigations for hypercoagulable disorders were within normal limits. Malignancy was ruled out by positron emission tomography-CT (PET-CT). Doppler studies of the lower-extremity veins were normal. A diagnosis of subacute pulmonary thromboembolism was made on the basis of the patient's clinical presentation and imaging results, and we began anticoagulation with heparin. Thrombolytic therapy was not started because of her stable hemodynamic condition.Subsequently, the patient was placed on warfarin therapy for 3 months and monitored. Her echocardiogram revealed a dilated RV with reduced systolic function and moderate tricuspid regurgitation, no decrease in the size of the mass, and normal left ventricular function. Her sPAP was 122 mmHg. Computed tomograms of her chest showed web-like filling defects in the pulmonary vasculature on the right and left lower lobes, arising from a pulmonary-valve condition that was consistent with

show abstract

“…Pulmonary artery intimal sarcoma has similar clinical manifestations to pulmonary embolism (including chest pain, difficulty breathing, cough, chest tightness, oedema and hemoptysis), which leads to an initially false diagnosis and delayed interventions (5). Percutaneous intravascular biopsy using a bronchoscopy forceps or ultrasound-guided transbronchial needle aspiration may obtain an early definitive diagnosis (2,3). The prognosis of this tumor is extremely poor, with a survival time of 12-18 months subsequent to the appearance of clinical symptoms (6).…”

Section: Introductionmentioning

confidence: 99%

Pulmonary artery intimal sarcoma misdiagnosed as pulmonary embolism: A case report

Jiang

Zeng

et al. 2017

Oncology Letters

View full text Add to dashboard Cite

Abstract. Intimal sarcoma of the pulmonary artery is rare, but false diagnosis of this sarcoma as pulmonary embolism is not infrequent. The present study reports a case of pulmonary artery intimal sarcoma misdiagnosed as pulmonary artery embolism in a 37-year-old female patient. The patient was admitted to the cardiac intensive care unit of Guangdong General Hospital (Guangzhou, China) with the complaint of progressive exertional dyspnea over the past two years. Multi-slice spiral computed tomography, transthoracic echocardiography, right-heart catheterization and cardiac magnetic resonance imaging revealed mimicking severe pulmonary embolism in the pulmonary trunk and right pulmonary artery, with symptoms including chest pain, cough and breathing difficulties. In addition, positron emission tomography-computed tomography results did not identify increased 18F-fluorodeoxyglucose uptake and failed to distinguish whether the mass was a thrombus or a malignancy. The patient was diagnosed with pulmonary embolism and a subcutaneous injection of 5,000 AxaIU enoxaparin sodium (100 AXAIU/kg) was administered every 12 h, but no improvement was achieved after 5 days of treatment. Finally, pulmonary endarterectomy was performed to relieve the worsening clinical symptoms. The clinicopathological diagnosis was pulmonary artery intimal sarcoma with poor clinical outcome. For this type of tumor with fatal prognosis, early and correct diagnosis may lead to appropriate intervention and prolong survival. IntroductionSince Moritz Mandelstamm first described intimal sarcoma of the pulmonary artery in 1923 (1), this tumor has been an uncommon but aggressive malignant tumor among the pulmonary artery, and may develop a fatal course leading to heart failure without treatment (2,3). The incidence is reported to be 0.001%, but this incidence appears to be inaccurate due to the challenging (4). Pulmonary artery intimal sarcoma has similar clinical manifestations to pulmonary embolism (including chest pain, difficulty breathing, cough, chest tightness, oedema and hemoptysis), which leads to an initially false diagnosis and delayed interventions (5). Percutaneous intravascular biopsy using a bronchoscopy forceps or ultrasound-guided transbronchial needle aspiration may obtain an early definitive diagnosis (2,3). The prognosis of this tumor is extremely poor, with a survival time of 12-18 months subsequent to the appearance of clinical symptoms (6). Surgical resection is the main treatment to prolong survival, which includes pulmonary endarterectomy (PEA), lobectomy and pneumonectomy (7). Combination doxorubicin and ifosfamide as first-line chemotherapy has been reportedto be transiently effective in the inhibition of rapid enlargement of the tumor. However, the effects of adjuvant radiation, radiofrequency ablation and chemotherapy subsequent to surgery remain unclear, and further studies are warranted (5). Case reportA 37-year-old woman was admitted to Guangdong General Hospital (Guangzhou, China) on December 25, 2014 with the complaint o...

show abstract

Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma†

Abstract: The prognosis of this very infrequent disease remains poor. Bilateral pulmonary endarterectomy may yield significant survival rates because it provides completeness of resection without sacrificing the pulmonary vascular bed.

Cited by 127 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

Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism

Pulmonary artery intimal sarcoma misdiagnosed as pulmonary embolism: A case report

Contact Info

Product

Resources

About