Pulmonary capillary hemangiomatosis (PCH)
Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 8 January 2013, last major update September 2012
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● Proliferation of benign-appearing capillaries in alveolar septa that appear to compress pulmonary veins; may represent a secondary angioproliferative process of veno-occlusive disease caused by postcapillary obstruction rather than a distinct entity (Am J Surg Pathol 2006;30:850)
● May present with pulmonary hypertension or interstitial lung disease
● Mean age 30 years, range 2-71 years
● Poor prognosis
● Lung transplantation
● Proliferation of benign-appearing capillaries in alveolar septa that appear to compress pulmonary veins
● Also hemorrhage, hemosiderosis
● Vascular transformation of sinuses in lobar, hilar and mediastinal lymph nodes is common, with intra-sinusal hemorrhage, erythrophagocytosis, lymphoid follicular hyperplasia (Virchows Arch 2008;453:171)
A-small veins demonstrate marked myointimal thickening; adjacent alveolar septa are thickened by endothelial cell proliferation of pulmonary capillary hemangiomatosis; (B) CD31 highlights endothelial cell proliferation
● CD31, CD34
● Veno-occlusive disease: associated with PCH; eccentric intimal thickening of venules in lobular septa with some septal veins completely occluded, increased elastic fibers in venous media (highlighted with elastin stain); intimal fibrosis narrows and occludes the pulmonary veins, causing dilatation of capillaries
End of Lung tumor > Benign tumors > Pulmonary capillary hemangiomatosis
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