Primary cardiac and pericardial tumors are rare entities with an autopsy frequency of 0.001-0.03%. Metastases to the heart and pericardium are much more common than primary tumors. Malignant pericardial mesotheliomas account for up to 50% of primary pericardial tumors. We report the case of a 75-year-old woman with hypertension, dyslipidemia and atrial fibrillation who went to the emergency department due to nonspecific thoracic discomfort of over six hours duration associated with syncope. Physical examination revealed a low-amplitude arrhythmic pulse, no heart murmurs and no signs of pulmonary congestion. The ECG revealed atrial fibrillation with ST-segment elevation in V2-V6, I and aVL. The patient was transferred for emergent coronary angiography, which revealed a long stenosis in the mid-distal portion of the left anterior descending artery. The echocardiogram showed a large pericardial effusion with diffuse thickening of the myocardium. Due to worsening hemodynamics, cardiac rupture was suspected and the patient underwent urgent sternotomy and pericardiotomy with drainage of a large quantity of hematic fluid. The surgeons then identified a large, unresectable tumor occupying the distal half of the anterior portion of the heart. This is, to our knowledge, the first case report of primary pericardial mesothelioma presenting with suspected ST-elevation myocardial infarction. In this case, direct observation of the tumor led to biopsy and the final diagnosis. These are highly malignant tumors and when diagnosed are usually already at an advanced stage.
Nineteen children, who had been admitted to the Pediatric Hospital of Coimbra with severe meningococcal infection with DIC, were submitted to clinical and radiological examination, an average of 4.2 years after hospitalization, in order to demonstrate late skeletal lesions. Patellar dystrophy, to our knowledge previously unreported, was found in 4 children, all of whom had overlying cutaneous scarring.
Solitary Fibrous Tumor is a rare neoplasm derived from mesenchymal cell. To achieve a diagnosis and to differentiate from others neoplasm immunohistochemical analysis is needed. The majority of these tumours are benign, and complete surgical resection remains the mainstay of therapy for both the benign and malignant variants. However, there are cases of local and distant recurrences, so patients should have a close follow-up and it is far incorrect to look at this lesion as definitely benign. The authors review clinical presentation, radiological features, histological findings, and treatment options for this tumor and report two clinical cases discussing the differences between them.
A arterite de Takayasu é uma doença inflamatória rara, idiopática, crónica e progressiva que causa estreitamento, oclusão e aneurismas das artérias sistémicas, afetando principalmente a aorta e seus ramos. O envolvimento neurológico surge numa minoria dos casos de arterite de Takayasu, e a ocorrência de síndromes neurológicas como primeira manifestação foram raramente relatados. Para o diagnóstico desta vasculite deve-se correlacionar os dados clínicos com os achados dos exames complementares, e uma vez feito o diagnóstico deve-se iniciar prontamente terapêutica a fim de minimizar a lesão de órgãos.
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