Cases reported "Sarcoma"

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1/21. Sarcoma of the main pulmonary artery: an unusual etiology for recurrent pulmonary emboli.

    We describe a case of primary pulmonary artery (PA) trunk spindle cell sarcoma in an 86 year old female presenting clinically with debilitating signs of recurrent pulmonary embolism. Further extensive work aroused suspicion for pulmonary artery malignancy. Palliative wide surgical resection, pulmonary artery tumor embolectomy and reconstruction of the proximal pulmonary artery and right ventricle outflow tract (RVOT) with bovine pericardial tissue were performed. She survived the procedure with an improved quality of life, but expired due to recurrence at 6 months postoperatively. Albeit uncommon, pulmonary artery sarcoma is nowadays a more frequently preoperatively diagnosed and surgically treated malignancy. With a modern low perioperative mortality, aggressive surgical resection remains as the single most effective modality for its treatment and can result in short term palliation in selected patients.
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ranking = 1
keywords = pulmonary embolism, embolism
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2/21. Case report of a patient with an intimal sarcoma of the pulmonary trunk presenting as a pulmonary embolism.

    A fatal case of an 89-year-old woman with an intimal sarcoma obstructing the pulmonary trunk and an open foramen ovale is presented. Clinical symptoms, physical examination and further evaluation originally raised suspicion of a pulmonary embolism. Recent classification systems, specific radiological and pathological characteristics of sarcomas of the pulmonary trunk are discussed.
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ranking = 5
keywords = pulmonary embolism, embolism
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3/21. pulmonary artery sarcoma: a case report of surgical cure and 5-year follow-up.

    pulmonary artery sarcoma is a rare tumor that is frequently misdiagnosed as chronic pulmonary embolism. With heightened clinical awareness and advancement in technology, the diagnosis is now increasingly being made preoperatively. Previous literature has described the disease to be uniformly fatal, with surgical resection as the single most effective modality for short-term palliation. We present the case of a patient in whom pulmonary artery sarcoma was diagnosed preoperatively and who underwent surgical resection with no evidence of recurrence during long-term follow-up, suggesting that early identification and aggressive surgical intervention has the potential to be curative.
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ranking = 1
keywords = pulmonary embolism, embolism
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4/21. Primary and secondary pulmonary artery neoplasia mimicking acute pulmonary embolism.

    Two cases of rare pulmonary neoplastic lesions (primary pulmonary artery sarcoma and pulmonary carcinosarcoma) directly involving the pulmonary artery and presenting with findings mimicking acute pulmonary embolism are discussed. Although this represents an unusual presentation for these two lesions, they should be included in the differential diagnosis of acute pulmonary embolism in patients presenting with somewhat atypical clinical, isotopic, and radiological features. Serial lung scans followed by angiography will prove useful in arriving at the correct diagnosis.
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ranking = 6
keywords = pulmonary embolism, embolism
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5/21. Tumor embolism as a cause of an unexpected death: a case report.

    The primary causes of deaths for individuals with rare cancers can be difficult to diagnose clinically. Often, the symptoms implicate a variety of factors, and an autopsy is thus required to obtain the correct diagnosis. This study analyzes the death of a 45-year-old woman who reportedly died from an acute pulmonary dysfunction. The patient had been treated with antibiotics for three months for intractable pneumonia. Suspicious coin lesions detected by chest X-ray prompted a clinical clarification; however, no final diagnosis was made. The autopsy revealed a bulky thyroid tumor with venous invasion, leading to a massive pulmonary tumor embolism. Furthermore, microscopy identified the tumor as a rare pleomorphic myxoid sarcoma. Thus, the patient died of a large pulmonary tumor embolism originating from this rare sarcoma, and not of acute pulmonary dysfunction of any other means.
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ranking = 0.25793040302289
keywords = embolism
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6/21. reproduction of features of the glucagonoma syndrome with continuous intravenous glucagon infusion as therapy for tumor-induced hypoglycemia.

    OBJECTIVE: To describe the adverse effects of continuous intravenous infusion of glucagon as therapy for tumor-induced hypoglycemia and to correlate these treatment-related effects with symptoms of endogenous hyper-glucagonemia. methods: We reviewed three cases in which patients received continuous glucagon therapy for tumor-induced hypoglycemia and experienced adverse side effects to the treatment. We noted that these adverse events were consistent with changes that are described in the literature as symptoms of the glucagonoma syndrome. RESULTS: Continuous intravenous glucagon infusion has evolved as a reliable and efficacious modality for the treatment of tumor-induced hypoglycemia. We report the adverse events of venous thromboembolism, necrolytic migratory erythema, and angular cheilitis in conjunction with continuous intravenous glucagon treatment. These complications resemble symptoms that characterize the human model of hyperglucagonemia--the glucagonoma syndrome--which is associated with hyperglucagonemia and alpha-islet cell neoplasms of the pancreas. CONCLUSION: Symptoms that characterize the islet cell neoplasm-related glucagonoma syndrome may develop in patients receiving an infusion of exogenous glucagon. This observation lends support to the suggestion that glucagon may have a direct, causative role.
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ranking = 4.1007637163594
keywords = thromboembolism, embolism
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7/21. Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature.

    The objectives of this study were to describe five cases involving primary tumors of the thoracoabdominal aorta and to review the pertinent literature. Between April 1990 and April 2000, we performed surgery on five patients with primary tumors of the aorta (PTA). There were three men and two women ranging in age from 37 to 65 years (mean, 49.8 years). The presenting manifestations were renovascular hypertension in four cases, including three associated with abdominal angina and lower extremity embolism in one case. In all patients aortograms identified atherosclerotic-like occlusive lesions in the thoracoabdominal aorta extending to the descending thoracic aorta in three cases, visceral arteries in four cases, and infrarenal aorta in one case. Preoperative histological diagnosis of PTA was achieved in two patients following open repair with placement of an aortoaortic graft in one case and peripheral embolectomy in one case. In two cases, diagnosis of PTA was strongly suspected before or during the procedure. In the remaining case, diagnosis was not achieved until the definitive histological report. In two patients surgical treatment was carried out with curative intent (aortic resection with graft replacement). In two cases surgical treatment was incomplete (endarterectomy of the aorta and visceral arteries). In the remaining case surgical treatment was purely palliative (aortic and superior mesenteric artery bypass). Histological findings demonstrated intimal-type sarcoma in two cases, leiomyosarcoma in one case, and angiosarcoma in one case. In the remaining case, histological analysis was unfeasible for technical reasons. One patient died due to massive cerebral embolism 2 days after surgical treatment involving revascularization of the aortic arch carried out with hypothermic circulatory arrest. One patient developed secondary paraplegia. All four patients who survived the immediate postoperative period died of tumor-related complications and cachexia at 5, 7, 16, and 24 months after the initial surgical procedure. The results of this small series as well as those of 130 previously reported cases confirm the extremely dismal prognosis of PTA. Mean overall survival for patients presenting PTA was less than 16 months. survival at 5 years was 8%. survival rates appear to be higher after surgical treatment and were significantly improved by adjuvant chemotherapy. The main factors correlated with poor prognosis were intimal type, involvement of the ascending aorta, aortic arch, or visceral aorta, and incomplete resection.
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ranking = 0.085976801007631
keywords = embolism
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8/21. 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 = 44.261957345108
keywords = pulmonary thromboembolism, thromboembolism, embolism
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9/21. Primary endothelial sarcoma of the thoracic aorta.

    Primary malignant tumors of the aorta are extremely rare. The case of a 64-year-old woman who presented with peripheral embolism to both femoropopliteal arteries is reported. The search for a source revealed a polypoid lesion severely narrowing the lumen of the distal thoracic aorta. Differential diagnosis included thrombus and primary aortic tumor. Extirpation of the tumorous lesion was performed. Histologic examination revealed intimal aortic sarcoma of endothelial cell origin. Although the liver was the only site of suspected metastases at the time of operation, during the 18-month follow-up until the patient's death, generalized metastatic spread had developed. This case report thus demonstrates the generally poor prognosis of this rare variety of aortic sarcoma, in particular when symptoms have already occurred.
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ranking = 0.042988400503816
keywords = embolism
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10/21. Antibody titers to desmogleins 1 and 3 in a patient with paraneoplastic pemphigus associated with follicular dendritic cell sarcoma.

    BACKGROUND: Most paraneoplastic pemphigus (PNP) cases reported to date have been associated with lymphoproliferative neoplasms. patients with PNP have autoantibodies against the plakin family (eg, envoplakin and periplakin). Antibodies against desmoglein 3 (Dsg3) and Dsg1, antigens for classic types of pemphigus, have also been reported to play an important role in the initial stage of PNP. OBSERVATIONS: We describe a patient with PNP associated with follicular dendritic cell sarcoma. Antibodies to envoplakin and periplakin were detected. When only mucosal lesions were observed at the early stage, the antibody to Dsg3 but not to Dsg1 was detected by enzyme-linked immunosorbent assay. After skin lesions appeared, antibodies to Dsg1 and Dsg3 were detected. These titers were elevated, with exacerbation of skin lesions. Although the patient received corticosteroid therapy, double-filtration plasmapheresis, and intravenous human immunoglobulin therapy after surgical resection of follicular dendritic cell sarcoma, she died of fungal infective lung embolisms. A direct immunofluorescence study of autopsy samples showed IgG deposition in the epidermis of the skin and oral mucosal membrane, but not in the lungs and kidneys and follicular dendritic cell sarcoma of the para-aortic area. CONCLUSION: In this patient with PNP and follicular dendritic cell sarcoma, there was an association between the clinical phenotype and the anti-Dsg antibody profile, as seen in pemphigus vulgaris.
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ranking = 0.042988400503816
keywords = embolism
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