Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism

Abstract

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

Single-Center Experience with Intimal Sarcoma, an Ultra-Orphan, Commonly Fatal Mesenchymal Malignancy

“…The prognosis of patients with pulmonary intimal sarcoma is poor, with a median OS of 12-18 months according to the literature [4,6,17,21]. Our findings are in accordance with this survival durations, with a mathematical median OS of 13 months.…”

“…Frequently it concerns extensive surgery involving vital structures, and vascular reconstructions are often needed. Immediate postoperative mortality is reported to be around 13-15%, and it is clear that the primary resection is rarely complete [6,[21][22][23]. In our series 10 patients had extensive primary surgery and all except 2 developed a local relapse or metastatic disease between 2 months and almost 6 years after local treatment.…”

Intimal sarcoma of the pulmonary artery presenting as pulmonary embolism

“…According to World Health Organization (WHO), there are three types of sarcoma of large blood vessels: angiosarcoma, leimyosarcoma and intimal sarcoma (3). Intimal sarcomas have a predominant intraluminal polipoyd growth pattern, often obstructing the lumen of the vessel of origin (2). Yet the clinical presentation is variable; patients usually have a long asymptomatic course.…”

“…These tumors arise from the intima and rarely extend to 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 surgery resection can be challenging (2). Prognosis, however, is still poor, with mean survival of around 10 months for those who undergo surgery, and 6 weeks for those who do not (4,5).…”

“…However, in the event of initial misdiagnosis and severe right ventricular dysfunction requiring immediate surgery, there might not be time for it (2). Adjuvant chemotherapy and radiation therapy are recommended for the eradication of any residual tumor foci (2).…”