Cases reported "Vascular Neoplasms"

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1/9. pulmonary artery leiomyosarcoma.

    pulmonary artery leiomyosarcoma is a rare but highly lethal disease, and can be mistaken for pulmonary thromboembolism. We report a case of pulmonary artery leiomyosarcoma managed with surgical resection, chemotherapy, and radiotherapy. A 57-year-old woman was admitted with complaints of aggravated dyspnea. She was initially treated with oxygen therapy and heparinization for a suspected pulmonary embolism. echocardiography revealed a dilated right atrium and ventricle and severe tricuspid regurgitation, with an estimated systolic right ventricular pressure of 95 mm Hg; a shadow of a mass in the main pulmonary artery was also noted. Right ventriculography revealed a filling defect, and to-and-fro motion of the mass in the main pulmonary artery. The left pulmonary artery was almost totally occluded by the mass. The patient's condition improved dramatically after palliative excision of the mass and patch reconstruction of the outflow tract of the right ventricle with a bicuspid xenograft. Pathologic examination of the mass revealed leiomyosarcoma. Chemotherapy and radiotherapy were subsequently administered and follow-up imaging studies 3 months postoperatively revealed no recurrence of the tumor. The patient remains well, more than 1 year after treatment. This report emphasises that pulmonary artery sarcoma should be considered in the differential diagnosis in cases of suspected pulmonary thromboembolism.
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ranking = 1
keywords = thromboembolism
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2/9. Primary leiomyosarcoma of the pulmonary artery mimicking massive pulmonary thromboembolism.

    A 57-year-old man with dyspnea and dry cough exhibited pulmonary embolism. Pulmonary arteriography demonstrated absent perfusion of the left main and the right upper and middle lobe pulmonary arteries. A diagnosis of chronic pulmonary thromboembolism was assumed and surgical thromboendarterectomy was attempted under standard cardiopulmonary bypass. At operation, a tumor had invaded far into both the right and left pulmonary arteries and radical resection was impossible. The final pathological diagnosis was primary leiomyosarcoma of the pulmonary artery. The patient refused any adjuvant therapy and died 63 days after the surgery. The clinical presentation of this case was similar to that of pulmonary thromboembolism and its diagnosis and treatment were very difficult.
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ranking = 3
keywords = thromboembolism
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3/9. Carcinomatous lymphangitis mimicking pulmonary thromboembolism.

    A 41-year-old woman was admitted with rapidly worsening dyspnea. echocardiography disclosed interventricular septal flattening and a markedly decreased left ventricle, although left ventricular contraction remained normal. Computed tomography of the chest demonstrated slightly dilated main pulmonary arteries and fine reticulonodular densities in the lung. Examination of a transbronchial lung biopsy specimen revealed carcinomatous lymphangitis, and the patient died 7 days after admission. The clinical presentation of this patient was difficult to discriminate from that seen with pulmonary thromboembolism.
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ranking = 2.5
keywords = thromboembolism
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4/9. leiomyosarcoma of the pulmonary artery--a diagnostic chameleon.

    A 60-year-old woman suffered from right-sided pain in the back and dyspnea suspicious for chronic pulmonary thromboembolism. Since computed tomography demonstrated a progressive filling defect within the left pulmonary artery, a transvenous biopsy was taken, which demonstrated malignant sarcoma. The patient underwent left-sided pneumonectomy with the aid of cardiopulmonary bypass. In case of a suspected 'chronic' pulmonary embolism with occlusion of a main pulmonary artery, as may be seen with imaging techniques, a sarcomatous disease should be ruled out, especially if there are no coagulation disorders, and the tumor obliterations progress in serial CT scans despite effective anticoagulation.
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ranking = 0.5
keywords = thromboembolism
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5/9. Primary leiomyosarcoma of the pulmonary artery: a diagnostic dilemma.

    Primary leiomyosarcoma of the pulmonary artery is a rare malignancy arising from the multipotential mesenchymal cell of the intima of the pulmonary artery. Due to its rarity and nonspecific clinical symptoms, the correct diagnosis and proper management are often delayed. Furthermore, it is frequently misdiagnosed as pulmonary embolism, mediastinal mass, pulmonary stenosis and lung cancer. Therefore, it is important to consider primary leiomyosarcoma of the pulmonary artery a possibility when a persistent filling defect is present in the pulmonary artery and there is no response to optimal anticoagulation treatment. Radiologic findings such as a unilateral mass continuously filling the pulmonary artery, inhomogenous enhancement, vascular distension, extravascular invasion into adjacent structure or uptake in the area of tumor on the FDG-PET can be helpful when differentiating pulmonary artery sarcoma (PAS) from chronic thromboembolism.
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ranking = 0.5
keywords = thromboembolism
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6/9. pulmonary artery leiomyosarcoma with thyroid metastases.

    Primary pulmonary artery sarcomas are uncommon and usually fatal tumours. The diagnosis of these tumours is delayed in most cases as they are mistaken for pulmonary thromboembolism. We present a fatal case of a woman referred to us five months after a primary diagnosis of pulmonary thromboembolism, due to an increase in dyspnea and presence of hemoptysis despite having undergone anticoagulant treatment. On the basis of the findings obtained by computed tomography, echocardiogram and MRI, a mass arising from the pulmonary trunk was evidenced, that suggested other diagnostic hypotheses. The worsening of patient's conditions did not allow an endovascular catheter biopsy and diagnosis was made at autopsy. The mass was a leiomyosarcoma of the pulmonary artery with thyroid metastases, which is an uncommon findings.
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ranking = 1
keywords = thromboembolism
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7/9. A case of pulmonary artery intimal sarcoma diagnosed with multislice CT scan with 3D reconstruction.

    pulmonary artery intimal sarcoma is a rare highly lethal disease, with additional retrograde extension to pulmonic valve and right ventricle being an extremely rare condition. It is frequently mistaken for pulmonary thromboembolism. We report a case of 64-year-old woman with progressive dyspnea initially suspected and treated for pulmonary thromboembolism. Her helical chest CT scan with 3 dimensional (3D) reconstruction combined with echocardiography revealed a compacting main pulmonary artery mass extending to the right ventricular outflow tract and the right pulmonary artery. After excision of the mass, the patient's condition improved dramatically, and the pathologic findings revealed pulmonary intimal sarcoma. This report emphasizes that helical chest CT with 3D reconstruction can be an important tool to differentiate the characteristics of pulmonary artery lesions, such as intimal sarcoma and thromboembolism.
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ranking = 1.5
keywords = thromboembolism
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8/9. Surgical treatment of pulmonary artery primary myxosarcoma.

    Case of the 47-year-old man, clinically suffering from recurrent dyspnoea, cough, syncopes, haemoptysis, unsuccessfully treated with anticoagulant and fibrinolytic agents, is described. Large masses of a tumour, occupying nearly the whole lumen of pulmonary artery and extending to peripheral branches, were removed during surgery. The tumour was histologically and immunohistochemically classified as myxosarcoma. Following surgery, the patient was asymptomatic, could do his original job (lorry-driver) for the next 14 months. He died 20 months after surgery, showing symptoms and signs of progressive right heart failure. At autopsy, the tumourous obliteration of the pulmonary artery and adjacent thrombosis were found. Primary pulmonary artery sarcoma should be considered in the differential diagnosis of recurrent pulmonary thromboembolism, especially when no peripheral vein thrombosis could be found.
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ranking = 0.5
keywords = thromboembolism
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9/9. Primary choriocarcinoma of the pulmonary artery mimicking pulmonary embolism.

    Due to their rarity, primary tumours of the pulmonary arteries are often incorrectly diagnosed as more common diseases such as pulmonary thromboembolism and are seldom diagnosed during a patient's lifetime. Surgery or potentially curative chemotherapy may therefore be withheld. We report a patient with a primary choriocarcinoma of the left pulmonary artery, which was first suspected on a CT scan. The neoplastic nature of the obstruction of the pulmonary arteries was confirmed by positron emission tomography.
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ranking = 0.5
keywords = thromboembolism
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