DOI: 10.1148/rg.273065194
Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis1
Aletta Ann Frazier, MD,
Teri J. Franks, MD,
Tan-Lucien H. Mohammed, MD, FCCP,
Irem H. Ozbudak, MD, and
Jeffrey R. Galvin, MD
1 From the Departments of Radiologic Pathology (A.A.F., J.R.G.) and Pulmonary and Mediastinal Pathology (T.J.F.), Armed Forces Institute of Pathology, 14th St and Alaska Ave NW, Washington, DC 20306; Department of Diagnostic Radiology, University of Maryland School of Medicine, Baltimore, Md (A.A.F., J.R.G.); Section of Thoracic Imaging, Division of Radiology, Cleveland Clinic Foundation, Cleveland, Ohio (T.-L.H.M.); and Department of Pathology, Akdeniz University School of Medicine, Antalya, Turkey (I.H.O.). Received December 6, 2006; revision requested December 18 and received January 18, 2007; accepted January 25. All authors have no financial relationships to disclose.
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Figure 1a. PVOD in a 20-year-old man. (a) Posteroanterior chest radiograph demonstrates a prominent main pulmonary artery (arrow), fissural thickening, and Kerley B lines. (b) Radiograph collimated to the left lower lobe helps confirm the presence of Kerley B lines (arrowheads).
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Figure 1b. PVOD in a 20-year-old man. (a) Posteroanterior chest radiograph demonstrates a prominent main pulmonary artery (arrow), fissural thickening, and Kerley B lines. (b) Radiograph collimated to the left lower lobe helps confirm the presence of Kerley B lines (arrowheads).
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Figure 2a. PVOD in a 56-year-old woman. (a) Radiograph collimated to the right lower lobe demonstrates numerous Kerley B lines (arrow). (b) Axial computed tomographic (CT) image (lung window level) collimated to the right lower lobe helps confirm smoothly thickened interlobular septa (arrowheads) and geographic ground-glass opacities.
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Figure 2b. PVOD in a 56-year-old woman. (a) Radiograph collimated to the right lower lobe demonstrates numerous Kerley B lines (arrow). (b) Axial computed tomographic (CT) image (lung window level) collimated to the right lower lobe helps confirm smoothly thickened interlobular septa (arrowheads) and geographic ground-glass opacities.
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Figure 3a. PVOD in a 17-year-old girl. (a) Coronal reformatted CT scan (lung window level) reveals widespread septal lines (arrowheads) and diffuse, ill-defined ground-glass nodules (arrows). (b) Photograph of a cut coronal section of the right lung reveals septal prominence (curved arrow) and ill-defined reddish-tan parenchymal nodules (straight arrow). (c) Axial CT image (mediastinal window level) reveals a thickened anterior wall of the right ventricle (curved arrow), a straightened interventricular septum (straight arrow), and a dilated right atrium compatible with cor pulmonale.
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Figure 3b. PVOD in a 17-year-old girl. (a) Coronal reformatted CT scan (lung window level) reveals widespread septal lines (arrowheads) and diffuse, ill-defined ground-glass nodules (arrows). (b) Photograph of a cut coronal section of the right lung reveals septal prominence (curved arrow) and ill-defined reddish-tan parenchymal nodules (straight arrow). (c) Axial CT image (mediastinal window level) reveals a thickened anterior wall of the right ventricle (curved arrow), a straightened interventricular septum (straight arrow), and a dilated right atrium compatible with cor pulmonale.
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Figure 3c. PVOD in a 17-year-old girl. (a) Coronal reformatted CT scan (lung window level) reveals widespread septal lines (arrowheads) and diffuse, ill-defined ground-glass nodules (arrows). (b) Photograph of a cut coronal section of the right lung reveals septal prominence (curved arrow) and ill-defined reddish-tan parenchymal nodules (straight arrow). (c) Axial CT image (mediastinal window level) reveals a thickened anterior wall of the right ventricle (curved arrow), a straightened interventricular septum (straight arrow), and a dilated right atrium compatible with cor pulmonale.
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Figure 5a. PCH in a 22-year-old woman. (a) Posteroanterior chest radiograph shows a prominent central pulmonary artery (arrow) and faint nodular opacities, best seen in the lung bases. (b) Radiograph collimated to the right lower lobe reveals widespread, poorly circumscribed nodular opacities (arrowheads). (c) Axial CT image (lung window level) collimated to the right lower lobe shows well-circumscribed ground-glass nodules (arrowheads) and no septal lines. (d) Photograph of the visceral pleural surface reveals multiple petechial-appearing nodules (arrow), just visible beneath the pleura, that actually represent the angiomatous lesions of PCH. (e) Low-power photomicrograph (original magnification, x1; hematoxylineosin [H-E] stain) shows multiple discrete parenchymal nodules (arrowheads). (f) Low-power photomicrograph (original magnification, x4; H-E stain) shows a discrete parenchymal nodule (arrowheads) abutting the visceral pleura.
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Figure 5b. PCH in a 22-year-old woman. (a) Posteroanterior chest radiograph shows a prominent central pulmonary artery (arrow) and faint nodular opacities, best seen in the lung bases. (b) Radiograph collimated to the right lower lobe reveals widespread, poorly circumscribed nodular opacities (arrowheads). (c) Axial CT image (lung window level) collimated to the right lower lobe shows well-circumscribed ground-glass nodules (arrowheads) and no septal lines. (d) Photograph of the visceral pleural surface reveals multiple petechial-appearing nodules (arrow), just visible beneath the pleura, that actually represent the angiomatous lesions of PCH. (e) Low-power photomicrograph (original magnification, x1; hematoxylineosin [H-E] stain) shows multiple discrete parenchymal nodules (arrowheads). (f) Low-power photomicrograph (original magnification, x4; H-E stain) shows a discrete parenchymal nodule (arrowheads) abutting the visceral pleura.
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Figure 5c. PCH in a 22-year-old woman. (a) Posteroanterior chest radiograph shows a prominent central pulmonary artery (arrow) and faint nodular opacities, best seen in the lung bases. (b) Radiograph collimated to the right lower lobe reveals widespread, poorly circumscribed nodular opacities (arrowheads). (c) Axial CT image (lung window level) collimated to the right lower lobe shows well-circumscribed ground-glass nodules (arrowheads) and no septal lines. (d) Photograph of the visceral pleural surface reveals multiple petechial-appearing nodules (arrow), just visible beneath the pleura, that actually represent the angiomatous lesions of PCH. (e) Low-power photomicrograph (original magnification, x1; hematoxylineosin [H-E] stain) shows multiple discrete parenchymal nodules (arrowheads). (f) Low-power photomicrograph (original magnification, x4; H-E stain) shows a discrete parenchymal nodule (arrowheads) abutting the visceral pleura.
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Figure 5d. PCH in a 22-year-old woman. (a) Posteroanterior chest radiograph shows a prominent central pulmonary artery (arrow) and faint nodular opacities, best seen in the lung bases. (b) Radiograph collimated to the right lower lobe reveals widespread, poorly circumscribed nodular opacities (arrowheads). (c) Axial CT image (lung window level) collimated to the right lower lobe shows well-circumscribed ground-glass nodules (arrowheads) and no septal lines. (d) Photograph of the visceral pleural surface reveals multiple petechial-appearing nodules (arrow), just visible beneath the pleura, that actually represent the angiomatous lesions of PCH. (e) Low-power photomicrograph (original magnification, x1; hematoxylineosin [H-E] stain) shows multiple discrete parenchymal nodules (arrowheads). (f) Low-power photomicrograph (original magnification, x4; H-E stain) shows a discrete parenchymal nodule (arrowheads) abutting the visceral pleura.
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Figure 5e. PCH in a 22-year-old woman. (a) Posteroanterior chest radiograph shows a prominent central pulmonary artery (arrow) and faint nodular opacities, best seen in the lung bases. (b) Radiograph collimated to the right lower lobe reveals widespread, poorly circumscribed nodular opacities (arrowheads). (c) Axial CT image (lung window level) collimated to the right lower lobe shows well-circumscribed ground-glass nodules (arrowheads) and no septal lines. (d) Photograph of the visceral pleural surface reveals multiple petechial-appearing nodules (arrow), just visible beneath the pleura, that actually represent the angiomatous lesions of PCH. (e) Low-power photomicrograph (original magnification, x1; hematoxylineosin [H-E] stain) shows multiple discrete parenchymal nodules (arrowheads). (f) Low-power photomicrograph (original magnification, x4; H-E stain) shows a discrete parenchymal nodule (arrowheads) abutting the visceral pleura.
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Figure 5f. PCH in a 22-year-old woman. (a) Posteroanterior chest radiograph shows a prominent central pulmonary artery (arrow) and faint nodular opacities, best seen in the lung bases. (b) Radiograph collimated to the right lower lobe reveals widespread, poorly circumscribed nodular opacities (arrowheads). (c) Axial CT image (lung window level) collimated to the right lower lobe shows well-circumscribed ground-glass nodules (arrowheads) and no septal lines. (d) Photograph of the visceral pleural surface reveals multiple petechial-appearing nodules (arrow), just visible beneath the pleura, that actually represent the angiomatous lesions of PCH. (e) Low-power photomicrograph (original magnification, x1; hematoxylineosin [H-E] stain) shows multiple discrete parenchymal nodules (arrowheads). (f) Low-power photomicrograph (original magnification, x4; H-E stain) shows a discrete parenchymal nodule (arrowheads) abutting the visceral pleura.
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Figure 6a. PCH in a 27-year-old woman. (a) Coronal reformatted CT scan (lung window level) shows diffuse, ill-defined, ground-glass nodules (arrowhead) and no evidence of interlobular septal thickening. (b) Photograph of a cut coronal section of the lung reveals multiple parenchymal nodules that appear hemorrhagic (arrow).
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Figure 6b. PCH in a 27-year-old woman. (a) Coronal reformatted CT scan (lung window level) shows diffuse, ill-defined, ground-glass nodules (arrowhead) and no evidence of interlobular septal thickening. (b) Photograph of a cut coronal section of the lung reveals multiple parenchymal nodules that appear hemorrhagic (arrow).
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Figure 7a. Microscopic features of PVOD. (a) Medium-power photomicrograph (original magnification, x20; H-E stain) of a parenchymal vein demonstrates narrowing of the lumen by loose, edematous intimal fibrous tissue that has been recanalized to form three channels. (b) Medium-power photomicrograph (original magnification, x10; H-E stain) demonstrates a fibrotic interlobular septum containing a vein whose lumen is occluded by dense, collagen-rich fibrous tissue (arrowhead).
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Figure 7b. Microscopic features of PVOD. (a) Medium-power photomicrograph (original magnification, x20; H-E stain) of a parenchymal vein demonstrates narrowing of the lumen by loose, edematous intimal fibrous tissue that has been recanalized to form three channels. (b) Medium-power photomicrograph (original magnification, x10; H-E stain) demonstrates a fibrotic interlobular septum containing a vein whose lumen is occluded by dense, collagen-rich fibrous tissue (arrowhead).
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Figure 8a. Microscopic features of PVOD. (a) Medium-power photomicrograph (original magnification, x10; H-E stain) demonstrates an edematous interlobular septum (*) that contains a dilated lymphatic, the latter of which is identified by the presence of valves (arrowhead). (b) Medium-power photomicrograph (original magnification, x10; H-E stain) shows loop-like alveolar capillary dilatation (arrow) upstream from a narrowed vein (arrowhead). (c) High-power photomicrograph (original magnification, x40; H-E stain) demonstrates a muscularized arteriole composed of concentric layers of spindle-shaped smooth muscle cells that give the vessel a distinctly rounded configuration; typically, arterioles lack smooth muscle. (d) Medium-power photomicrograph (original magnification, x20; H-E stain) of a muscular pulmonary artery reveals medial hypertrophy (arrowhead) and, in this case, also intimal thickening (arrow).
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Figure 8b. Microscopic features of PVOD. (a) Medium-power photomicrograph (original magnification, x10; H-E stain) demonstrates an edematous interlobular septum (*) that contains a dilated lymphatic, the latter of which is identified by the presence of valves (arrowhead). (b) Medium-power photomicrograph (original magnification, x10; H-E stain) shows loop-like alveolar capillary dilatation (arrow) upstream from a narrowed vein (arrowhead). (c) High-power photomicrograph (original magnification, x40; H-E stain) demonstrates a muscularized arteriole composed of concentric layers of spindle-shaped smooth muscle cells that give the vessel a distinctly rounded configuration; typically, arterioles lack smooth muscle. (d) Medium-power photomicrograph (original magnification, x20; H-E stain) of a muscular pulmonary artery reveals medial hypertrophy (arrowhead) and, in this case, also intimal thickening (arrow).
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Figure 8c. Microscopic features of PVOD. (a) Medium-power photomicrograph (original magnification, x10; H-E stain) demonstrates an edematous interlobular septum (*) that contains a dilated lymphatic, the latter of which is identified by the presence of valves (arrowhead). (b) Medium-power photomicrograph (original magnification, x10; H-E stain) shows loop-like alveolar capillary dilatation (arrow) upstream from a narrowed vein (arrowhead). (c) High-power photomicrograph (original magnification, x40; H-E stain) demonstrates a muscularized arteriole composed of concentric layers of spindle-shaped smooth muscle cells that give the vessel a distinctly rounded configuration; typically, arterioles lack smooth muscle. (d) Medium-power photomicrograph (original magnification, x20; H-E stain) of a muscular pulmonary artery reveals medial hypertrophy (arrowhead) and, in this case, also intimal thickening (arrow).
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Figure 8d. Microscopic features of PVOD. (a) Medium-power photomicrograph (original magnification, x10; H-E stain) demonstrates an edematous interlobular septum (*) that contains a dilated lymphatic, the latter of which is identified by the presence of valves (arrowhead). (b) Medium-power photomicrograph (original magnification, x10; H-E stain) shows loop-like alveolar capillary dilatation (arrow) upstream from a narrowed vein (arrowhead). (c) High-power photomicrograph (original magnification, x40; H-E stain) demonstrates a muscularized arteriole composed of concentric layers of spindle-shaped smooth muscle cells that give the vessel a distinctly rounded configuration; typically, arterioles lack smooth muscle. (d) Medium-power photomicrograph (original magnification, x20; H-E stain) of a muscular pulmonary artery reveals medial hypertrophy (arrowhead) and, in this case, also intimal thickening (arrow).
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Figure 9a. Microscopic features of PCH. (a) Medium-power photomicrograph (original magnification, x20; H-E stain) demonstrates thickened and cellular alveolar walls due to capillary proliferation in PCH. (b) In contrast, this medium-power photomicrograph (original magnification, x20; H-E stain) shows the loop-like dilatation of capillaries in PVOD.
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Figure 9b. Microscopic features of PCH. (a) Medium-power photomicrograph (original magnification, x20; H-E stain) demonstrates thickened and cellular alveolar walls due to capillary proliferation in PCH. (b) In contrast, this medium-power photomicrograph (original magnification, x20; H-E stain) shows the loop-like dilatation of capillaries in PVOD.
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Figure 10a. Drawings of microscopic lung anatomy at the level of the secondary pulmonary lobule and an interlobular septum. (a) In normal hemodynamics, the centrilobular pulmonary artery (blue) enters the broad capillary network within the alveolar walls. The oxygenated blood then flows into interlobular septal veins (red). There is a lymphatic network (yellow) in both the pleura and interlobular septum. (b) In PVOD, the interlobular septal veins are occluded, the capillary network is secondarily dilated in a configuration of multiple vascular loops, the lymphatic channels are engorged, and the interlobular septum is edematous. (c) In PCH, a discrete area of capillary proliferation is present, typically without evidence of changes within the pulmonary veins or interlobular septum.
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Figure 10b. Drawings of microscopic lung anatomy at the level of the secondary pulmonary lobule and an interlobular septum. (a) In normal hemodynamics, the centrilobular pulmonary artery (blue) enters the broad capillary network within the alveolar walls. The oxygenated blood then flows into interlobular septal veins (red). There is a lymphatic network (yellow) in both the pleura and interlobular septum. (b) In PVOD, the interlobular septal veins are occluded, the capillary network is secondarily dilated in a configuration of multiple vascular loops, the lymphatic channels are engorged, and the interlobular septum is edematous. (c) In PCH, a discrete area of capillary proliferation is present, typically without evidence of changes within the pulmonary veins or interlobular septum.
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Figure 10c. Drawings of microscopic lung anatomy at the level of the secondary pulmonary lobule and an interlobular septum. (a) In normal hemodynamics, the centrilobular pulmonary artery (blue) enters the broad capillary network within the alveolar walls. The oxygenated blood then flows into interlobular septal veins (red). There is a lymphatic network (yellow) in both the pleura and interlobular septum. (b) In PVOD, the interlobular septal veins are occluded, the capillary network is secondarily dilated in a configuration of multiple vascular loops, the lymphatic channels are engorged, and the interlobular septum is edematous. (c) In PCH, a discrete area of capillary proliferation is present, typically without evidence of changes within the pulmonary veins or interlobular septum.
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Copyright © 2007 by the Radiological Society of North America.
