PULMONARY CAPILLARY HEMANGIOMATOSIS - A CLINICOPATHOLOGICAL REVIEW

Pulmonary capillary hemangiomatosis is a locally aggressive benign vas cular neoplasm of the lung characterized by the presence of numerous c ytologically benign thin-walled capillary-sized blood vessels prolifer ating diffusely through the pulmonary interstitium, in and around pulm onary vessels and airways. Pulmonary capillary hemangiomatosis is a ra re disease characterized by pulmonary hypertension and a slowly progre ssive clinical course; it is frequently misdiagnosed clinically as pri mary pulmonary hypertension and venoocclusive disease. The purpose of this review is to describe the clinical, radiologic, and histologic fe atures of this rare form of pulmonary vascular neoplasm, which may pre sent considerable diagnostic problems to both the clinician and the hi stopathologist. Fourteen cases of pulmonary capillary hemangiomatosis have been previously reported. In this review we describe the fourth c ase of pulmonary capillary hemangiomatosis in which the diagnosis was made antemortem, as well as the fourth to undergo lung transplantation .