Pulmonary Capillary Hemangiomatosis–Like Foci

Abstract

Pulmonary capillary hemangiomatosis (PCH) typically occurs in young patients who have signs and symptoms of pulmonary hypertension. It commonly is misdiagnosed in life as pulmonary veno-occlusive disease, and the correct diagnosis usually is not made until autopsy. Autopsy records, including reports, gross photographs, histologic slides, clinical histories, and radiographic images, were reviewed to identify cases with morphologic changes characteristic of PCH. The previous case reports describe PCH as a diffuse process throughout both lung fields. All patients were symptomatic, and most died of the disease. This article details 8 cases of PCH-like foci that were incidental findings at autopsy in which the patients did not have symptoms of pulmonary hypertension nor did PCH contribute in any way to death. This is the first case series that describes pathologic changes of PCH occurring in this setting, and we hope to provide more interest in PCH and its natural history.