Unusual thoracic manifestations of epithelioid hemangioendothelioma

Intravascular bronchioloalveolar tumors are now recognized as the pulmonary analogue of epithelioid hemangioendotheliomas of the somatic soft tissues. These low-grade endothelial sarcomas have a typical histologic appearance and usually present as bilateral pulmonary nodules in young women. The present report describes four unusual clinical manifestations of the epithelioid hemangioendothelioma: as an anterior mediastinal mass, as diffuse pleural thickening resembling malignant mesothelioma, as a metastatic carcinoma with cancerization of the lymphatics (lymphagitic carcinoma), and as a solitary peripheral calcified nodule. These anomalous clinical presentations correspond to unusual histologic appearances of the tumor at open-lung biopsy, and need to be recognized by both the clinician and the pathologist.