[Pulmonary capillary hemangiomatosis: a rare cause of pulmonary hypertension].
Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension characterized by capillary proliferation infiltrating the structures of the pulmonary parenchyma. Although veins are particularly involved, proliferation also affects bronchiolar, interstitial and other structures. We report a case of PCH in a 70-year-old man. Pulmonary artery hypertension was demonstrated by echocardiogram and angiography. Severe emphysema could be seen in a computed tomographic scan of the thorax, even though spirometric values indicated that airflow obstruction was mild. Dyspnea and respiratory insufficiency progressed with marked shunting until death. Tissue inspection at the autopsy revealed capillary proliferation in the alveolar walls with occasional oviform protrusions into air spaces or around small vessels and bronchioles. Endothelial cells in newly formed vessels were not atypical and mitosis was scarce; p53 expression was negative and Ki67 proliferation slight, indicating that PCH is not a neoplastic process as has sometimes been suggested.
Bandrés Gimeno
R
R
Servicios Anatomía Patológica. Complexo Hospitalario Xeral-Cíes. Vigo. La Coruna. Spain. rafaelbandres@yahoo.es
González-Carreró
J
J
Iglesias
B
B
Vicente
C
C
Torres Durán
M L
ML
D000368:Aged; D000792:Angiography; D001344:Autopsy; D004452:Echocardiography; D018324:Hemangioma, Capillary / Q000150:complications* / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000209:etiology*; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000150:complications* / Q000473:pathology; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000473:pathology; D014057:Tomography, X-Ray Computed