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Lung tumor

Benign tumors

Pulmonary capillary hemangiomatosis (PCH)


Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 8 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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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

Treatment
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● Lung transplantation

Micro description
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● 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)

Micro images
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Various images


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

Positive stains
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● CD31, CD34

Differential diagnosis
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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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