Pulmonary capillary hemangiomatosis-like foci - An autopsy study of 8 cases

Pulmonary capillary hemangiomatosis (PCH) typically occurs in young patient s who have signs and symptoms of pulmonary hypertension. It commonly is mis diagnosed in life as pulmonary veno-occlusive disease, and the correct diag nosis usually is not made until autopsy. Autopsy records, including reports , gross photographs, histologic slides, clinical histories, and radiographi c images, were reviewed to identify cases with morphologic changes characte ristic of PCH. The previous case reports describe PCH as a diffuse process throughout both lung fields. All patients were symptomatic, and most died o f the disease. This article details 8 cases of PCH-like foci that were inci dental findings at autopsy in which the patients did not have symptoms of p ulmonary hypertension nor did PCH contribute in any way to death. This is t he 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 h istory.