High resolution CT findings of pulmonary epithelioid hemangioendothelioma - Unusual manifestations in 2 cases

Pulmonary epithelioid hemangioendotheliomas (PEH), also known as intravascular sclerosing bronchoalveolar tumor, is a rare vascular tumor of the lung common among young women. Primitive lumena lined by single cells is the characteristic pathologic feature. The endothelial nature of these cells is confirmed by positive staining with factor VIII and CD34. PEH usually presents as single or multiple pulmonary nodules. The present report describes high resolution CT (HRCT) findings of 2 cases with unusual manifestations of PEH. One case was a 54-year-old woman with multiple pulmonary nodules with irregular thickening of both the bronchovascular bundles and perilobular structures, representing intensive lymphangitic spread on HRCT. The other was an 18-year-old woman who had multiple minute peripheral nodules in the lungs bilaterally. These HRCT findings demonstrated the presence of tumor nodules in the lymphatic spaces, which is quite an unusual histologic presentation for this tumor. Both cases also showed hepatic lesions on abdominal CT; the former showing hypoattenuating masses with coarse calcifications and the latter showing multiple tiny calcifications in the hepatic parenchyma. Recognition of these features in the appropriate clinical setting may allow the clinician and the pathologist to consider this rare tumor.