Clinicopathologic characteristics of primary pleural epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare vascular-derived malignancy. Within the thorax, EHE originating in the pleura is less often reported than EHE originating in the lung. Pulmonary EHE has been referred to as a borderline or low-grade malignancy that exhibits an unpredictable course. It often is associated with a long life expectancy and occasional spontaneous remission. Because the natural history of pleural EHE is less well understood, a literature review was conducted to investigate the characteristics of the disease. Twenty-two cases of pleural EHE were identified. The disease occurred more often in men than women, appeared in the right thorax more often than the left, exhibited a virulent course, was usually refractory to systemic therapy, and was often associated with a short survival. These characteristics differ substantially from those of pulmonary EHE and suggest that the clinician should approach pleural EHE anticipating an especially aggressive malignancy which likely will not respond to current therapeutic measures.