A case of pulmonary epithelioid hemangioendothelioma / 대한내과학회지

ABSTRACT

Epithelioid hemangioendothelioma(EH) is a rare vascular tumor of low-grade malignancy. It was previously described as intravascular bronchioloalveolar tumor (IVBAT). But electron microscopic study and immunohistochemical staining results have proved endothelial origin. Patients are usually asymptomatic and the tumors are found incidentally on routine chest X-rays. Most patients show a slowly progressive clinical course, even though some patients occasionally die as results of respiratory failure or extrathoracic complications. Confirmative diagnosis is made through thoracoscopic or open-lung biopsy. There is still no effective therapeutic modality for pulmonary EH. We have experienced a 55-year-old woman whose simple chest x-ray film revealed bilateral multiple small nodules. The nodules were histopathologically and immunohistochemically diagnosed as primary epithelioid hemangioendothelioma of the lung. The possibility of metastatic disease was excluded thorough clinical, laboratory and radiological studies. Comparison of radiographic chest film taken 3 years ago showed no significant progression of the pulmonary nodular lesions. Conservative management was instituted and careful 4 months follow-up showed no significant changes.