What Looks Like a Clot But Is Not a Clot?: Cardiac Leiomyosarcoma Mimicking Pulmonary Embolism.
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ABSTRACT:
SUBMITTER: Leiva O
PROVIDER: S-EPMC8299237 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.
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Project description:Pulmonary embolism (PE) is a common finding in patients with underlying malignancy and is the commonest cause of acute cor pulmonale. A 65-year-old woman with a background of non-small-cell lung cancer presented to the emergency department with nausea and vomiting after starting erlotinib; she was pyrexial and had raised C-reactive protein. Despite aggressive fluid resuscitation and antibiotics the patient remained tachycardic, hypotensive, profoundly hypoxic and had a persistent raised jugular venous pulse. Massive PE was therefore suspected. A bedside echocardiogram demonstrated a dilated right ventricle and evidence of pulmonary hypertension. A CT pulmonary angiogram excluded a PE but revealed progression of the right hilar tumour causing complete obstruction of the right upper and middle lobe pulmonary arteries. This case highlights an important differential diagnosis when assessing patients with an underlying intrathoracic tumour with findings suggestive of PE and the importance of obtaining further imaging before considering thrombolysis.
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Project description:It is unclear whether thrombus location in pulmonary arteries is associated with particular clot characteristics. We assessed 156 patients following either central or peripheral pulmonary embolism (PE). Plasma clot lysis time, the rate of D-dimer release from plasma clots (D-Drate) with the maximum D-dimer concentration achieved (D-Dmax), as well as fibrin formation on turbidimetry, plasma clot permeation, thrombin generation, and fibrinolytic parameters were measured 3-6 months after PE. Patients following central PE (n?=?108, 69.3%) were more likely smokers (38.9% vs 18.8%; p?=?0.01), less likely carriers of factor XIII Val34Leu allele (40.7% vs 62.5%, p?=?0.01), exhibited 16.7% higher D-Drate and 12.7% higher tissue plasminogen activator antigen (tPA:Ag) compared with peripheral PE (p?=?0.02 and p?<?0.0001, respectively). Saddle PE patients (n?=?31, 19.9%) had 11.1% higher D-Drate and 7.3% higher D-Dmax compared with central PE (both p?<?0.05). Twenty-three recurrent PE episodes, including 15 central episodes, during a median follow-up of 52.5 months were recorded. Plasma D-dimer and tPA:Ag were independent predictors for central recurrent PE, whereas D-Drate and peak thrombin predicted peripheral recurrent PE. Plasma clots degradation is faster in patients following central PE compared with peripheral PE and fibrinolysis markers might help to predict a type of recurrent PE.
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Project description:BackgroundStachybotrys microspora triprenyl phenols (SMTPs) are a novel family of small molecules that enhance both activation and fibrin-binding of plasminogen. While their effects on fibrinolysis have been characterized in vitro, little is known about their activity in vivo with respect to plasminogen activation and blood clot clearance.ResultsTo select a potent SMTP congener for the evaluation of its action in vitro and in vivo, we tested several SMTP congeners with distinct structural properties for their effects on plasminogen activation. As a result, SMTP-7 (orniplabin) was found to have distinguished activity. Several lines of biochemical evidence supported the idea that SMTP-7 acted as a plasminogen modulator. SMTP-7 elevated plasma level of plasmin-?2-antiplasmin complex, an index of plasmin formation in vivo, 1.5-fold in mice after the intravenous injections at doses of 5 and 10 mg kg-1. In a rat pulmonary embolism model, SMTP-7 (5 mg kg-1) enhanced the rate of clot clearance ~3-fold in the absence of exogenous plasminogen activator. Clot clearance was enhanced further by 5 mg kg-1 of SMTP-7 in combination with single-chain urokinase-type plasminogen activator.ConclusionsOur results show that SMTP-7 is a superior plasminogen modulator among the SMTP family compounds and suggest that the agent enhances plasmin generation in vivo, leading to clearance of thrombi in a model of pulmonary embolism.
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Project description:Pulmonary artery sarcoma (PAS) is a rare and aggressive mesenchymal tumor that often mimics thromboembolic disease. Due to its rare and fatal nature, patients are often underdiagnosed or misdiagnosed. There is still no consensus regarding the diagnosis and treatment of PAS. We present a case of a 63 year old male misdiagnosed with pulmonary thromboembolism who received anticoagulant therapy. 18FDG positron emission tomography (PET) integrated with computed tomography (PET/CT) and subsequent surgery led to the final diagnosis of PAS. Whole exome sequencing of the tissue identified the genetic alterations profile of PAS: copy number variation (CNV) of KIT and mutations of TP53, PIK3CA, IL7R and ATR. Treated with chemotherapy followed by anlotinib, the patient's survival time was 8 months after firm diagnosis. To our knowledge, anlotinib used as a treatment for PAS has not been reported.
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Project description:PurposeTo assess the correlation between volumetric measurements of clot, semiquantitative clot burden indexes, and signs of right heart dysfunction at computed tomographic (CT) pulmonary angiography in patients with acute pulmonary embolism (PE) and to determine whether clot burden and signs of right heart dysfunction are associated with short-term mortality.Materials and methodsThis retrospective study was institutional review board approved and HIPAA compliant. CT pulmonary angiographic studies (January 2007 through December 2007) with findings positive for PE were retrieved. Two readers evaluated signs of right heart dysfunction at CT pulmonary angiography, measured clot volume using a dedicated software program, and assessed clot burden using semiquantitative scores (Qanadli and Mastora). Spearman rank coefficient was used to investigate correlation between clot burden measures and signs of right heart dysfunction. Uni- and multivariate analyses were used to test association between CT pulmonary angiographic findings and short-term mortality.ResultsA total of 635 CT pulmonary angiographic studies from 635 patients (304 men, 331 women; mean age, 59 years) were included; 39 (6%) patients died within 30 days. Clot volume was strongly correlated with Qanadli score (ρ=0.841, P<.01) and Mastora score (ρ=0.863, P<.01) and moderately correlated (ρ=0.378, P<.01) with the ratio of right ventricle diameter to left ventricle diameter (RV/LV ratio). Among the pulmonary angiographic signs, only increase in RV/LV ratio (cut-off value, 1.0) was independently associated with short-term mortality in multivariate analysis.ConclusionClot volume strongly correlated with semiquantitative CT scores of clot burden, and greater clot volume was associated with higher incidence of right heart dilatation. Increase in RV/LV ratio was associated with short-term mortality; however, measures of clot burden were not.
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Project description:Pulmonary embolism is a common cardiovascular emergency, but it is still often misdiagnosed due to its unspecific clinical symptoms. Elevated troponin concentrations are associated with greater morbidity and mortality in patients with pulmonary embolism. Right ventricular ischemia due to increased right ventricular afterload is believed to be underlying mechanism of elevated troponin values in acute pulmonary embolism, but a paradoxical coronary artery embolism through opened intra-atrial communication is another possible explanation as shown in our case report.
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