[Pulmonary epithelioid hemangioendothelioma: a clinicopathological analysis]

To report 3 cases of pulmonary epithelioid haemangioendothelioma (PEH) and therefore to improve the understanding of this tumor.

The clinical pathological features of 3 cases of PEH were described and related literatures were reviewed.

The etiology of this rare disease remained unknown. Symptoms were scanty and usually mild. Chest radiograph or computed tomography usually revealed multiple bilateral pulmonary nodules. Histologically, crown-like clusters of epithelioid tumor cells or spindle cells were filled in the alveoli at the periphery of the tumor nodules, while the central part of the nodules contained myxoid to hyaline matrix. Tumor cells generally lacked pleomorphism, mitotic activity and necrosis. They were immunohistochemically positive for CD(31) and CD(34). CK staining was positive in some cases. There was no effective treatment for this disease and its prognosis was unpredictable.

PEH is a low grade malignancy and represents a distinct clinical pathological entity. It is rare and often misdiagnosed as other pulmonary diseases.