DOI: 10.1148/rg.274075014
Pediatric Orbit Tumors and Tumorlike Lesions: Neuroepithelial Lesions of the Ocular Globe and Optic Nerve1
Ellen M. Chung, LTC, MC, USA,
Charles S. Specht, MD, and
Jason W. Schroeder, LT, MC, USN
1 From the Department of Radiologic Pathology (E.M.C.) and Ophthalmic Pathology Section, Department of Neuropathology (C.S.S.), Armed Forces Institute of Pathology, Alaska and Fern streets NW, Washington, DC 20306-6000; and the National Capitol Radiology Consortium, National Naval Medical Center, Bethesda, Md, and Walter Reed Army Medical Center, Washington, DC (J.W.S.). Received January 31, 2007; revision requested April 3 and received May 4; accepted May 4. All authors have no financial relationships to disclose.
View larger version (81K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1. Leukocoria in a 2-year-old boy with right retinoblastoma. In the right eye, the normal red pupillary reflex has been replaced by a grayish white reflex. The left eye shows the normal red reflex.
|
|
View larger version (130K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2a. Endophytic retinoblastoma in a 2-year-old boy with left leukocoria who had impaired vision in the left eye at ophthalmologic examination. (a) Photograph of the sectioned gross specimen shows a pinkish white mass in the posterior globe that abuts the retina (arrowhead) and has a nodular cut surface. * = lens. (b) Axial unenhanced computed tomographic (CT) image shows the nodular hyperattenuating mass (arrow), which occupies the posterior left globe and contains foci of calcification. (c) Axial T2-weighted magnetic resonance (MR) image shows that the tumor is fairly homogeneous and hypointense relative to the vitreous. (d, e) MR images (d obtained at a higher level than e) acquired with intravenous contrast material but without fat saturation show that the mass is hyperintense relative to the adjacent vitreous.
|
|
View larger version (87K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2b. Endophytic retinoblastoma in a 2-year-old boy with left leukocoria who had impaired vision in the left eye at ophthalmologic examination. (a) Photograph of the sectioned gross specimen shows a pinkish white mass in the posterior globe that abuts the retina (arrowhead) and has a nodular cut surface. * = lens. (b) Axial unenhanced computed tomographic (CT) image shows the nodular hyperattenuating mass (arrow), which occupies the posterior left globe and contains foci of calcification. (c) Axial T2-weighted magnetic resonance (MR) image shows that the tumor is fairly homogeneous and hypointense relative to the vitreous. (d, e) MR images (d obtained at a higher level than e) acquired with intravenous contrast material but without fat saturation show that the mass is hyperintense relative to the adjacent vitreous.
|
|
View larger version (100K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2c. Endophytic retinoblastoma in a 2-year-old boy with left leukocoria who had impaired vision in the left eye at ophthalmologic examination. (a) Photograph of the sectioned gross specimen shows a pinkish white mass in the posterior globe that abuts the retina (arrowhead) and has a nodular cut surface. * = lens. (b) Axial unenhanced computed tomographic (CT) image shows the nodular hyperattenuating mass (arrow), which occupies the posterior left globe and contains foci of calcification. (c) Axial T2-weighted magnetic resonance (MR) image shows that the tumor is fairly homogeneous and hypointense relative to the vitreous. (d, e) MR images (d obtained at a higher level than e) acquired with intravenous contrast material but without fat saturation show that the mass is hyperintense relative to the adjacent vitreous.
|
|
View larger version (112K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2d. Endophytic retinoblastoma in a 2-year-old boy with left leukocoria who had impaired vision in the left eye at ophthalmologic examination. (a) Photograph of the sectioned gross specimen shows a pinkish white mass in the posterior globe that abuts the retina (arrowhead) and has a nodular cut surface. * = lens. (b) Axial unenhanced computed tomographic (CT) image shows the nodular hyperattenuating mass (arrow), which occupies the posterior left globe and contains foci of calcification. (c) Axial T2-weighted magnetic resonance (MR) image shows that the tumor is fairly homogeneous and hypointense relative to the vitreous. (d, e) MR images (d obtained at a higher level than e) acquired with intravenous contrast material but without fat saturation show that the mass is hyperintense relative to the adjacent vitreous.
|
|
View larger version (114K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2e. Endophytic retinoblastoma in a 2-year-old boy with left leukocoria who had impaired vision in the left eye at ophthalmologic examination. (a) Photograph of the sectioned gross specimen shows a pinkish white mass in the posterior globe that abuts the retina (arrowhead) and has a nodular cut surface. * = lens. (b) Axial unenhanced computed tomographic (CT) image shows the nodular hyperattenuating mass (arrow), which occupies the posterior left globe and contains foci of calcification. (c) Axial T2-weighted magnetic resonance (MR) image shows that the tumor is fairly homogeneous and hypointense relative to the vitreous. (d, e) MR images (d obtained at a higher level than e) acquired with intravenous contrast material but without fat saturation show that the mass is hyperintense relative to the adjacent vitreous.
|
|
View larger version (106K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3a. Endophytic retinoblastoma in a 2-year-old girl with a white pupillary reflex in the right eye. (a) Photograph (hematoxylin-eosin [H-E] stain) of the whole-mount specimen shows a mass growing from the medial retina (arrowhead) toward the vitreous posterior to the lens (curved arrow). Calcifications are seen in the tumor (straight arrow). The tumor does not involve the optic nerve (*). (b) Unenhanced CT image shows the densely calcified mass abutting the retina in the medial right globe.
|
|
View larger version (155K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3b. Endophytic retinoblastoma in a 2-year-old girl with a white pupillary reflex in the right eye. (a) Photograph (hematoxylin-eosin [H-E] stain) of the whole-mount specimen shows a mass growing from the medial retina (arrowhead) toward the vitreous posterior to the lens (curved arrow). Calcifications are seen in the tumor (straight arrow). The tumor does not involve the optic nerve (*). (b) Unenhanced CT image shows the densely calcified mass abutting the retina in the medial right globe.
|
|
View larger version (112K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 4a. Retinoblastoma with an infiltrative growth pattern and optic nerve invasion in a 2-year-old adopted Chinese girl whose parents noted squinting of the right eye. Leukocoria was noted by the referring physician. (a) Photograph (H-E stain) of the whole-mount specimen shows diffuse thickening and nodularity of the detached retina (arrowheads) with extension of the tumor into the optic nerve (arrow). (b) Axial thin-section T2-weighted MR image obtained with fat saturation shows the diffusely nodular detached retina, which is hypointense relative to the vitreous. (c) Axial fat-saturated T1-weighted MR image, obtained by using a surface coil after intravenous administration of gadolinium contrast material, shows that the thickened detached retina is hyperintense relative to the vitreous. Invasion of the optic nerve is also evident (arrowhead).
|
|
View larger version (105K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 4b. Retinoblastoma with an infiltrative growth pattern and optic nerve invasion in a 2-year-old adopted Chinese girl whose parents noted squinting of the right eye. Leukocoria was noted by the referring physician. (a) Photograph (H-E stain) of the whole-mount specimen shows diffuse thickening and nodularity of the detached retina (arrowheads) with extension of the tumor into the optic nerve (arrow). (b) Axial thin-section T2-weighted MR image obtained with fat saturation shows the diffusely nodular detached retina, which is hypointense relative to the vitreous. (c) Axial fat-saturated T1-weighted MR image, obtained by using a surface coil after intravenous administration of gadolinium contrast material, shows that the thickened detached retina is hyperintense relative to the vitreous. Invasion of the optic nerve is also evident (arrowhead).
|
|
View larger version (101K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 4c. Retinoblastoma with an infiltrative growth pattern and optic nerve invasion in a 2-year-old adopted Chinese girl whose parents noted squinting of the right eye. Leukocoria was noted by the referring physician. (a) Photograph (H-E stain) of the whole-mount specimen shows diffuse thickening and nodularity of the detached retina (arrowheads) with extension of the tumor into the optic nerve (arrow). (b) Axial thin-section T2-weighted MR image obtained with fat saturation shows the diffusely nodular detached retina, which is hypointense relative to the vitreous. (c) Axial fat-saturated T1-weighted MR image, obtained by using a surface coil after intravenous administration of gadolinium contrast material, shows that the thickened detached retina is hyperintense relative to the vitreous. Invasion of the optic nerve is also evident (arrowhead).
|
|
View larger version (119K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 5a. Retinocytoma in a 3-year-old boy with left strabismus and leukocoria. (a) Photograph of the sectioned gross specimen shows a homogeneous white, smooth, biconvex mass (arrowhead) in the posterior pole that abuts the retina. (b) Axial unenhanced CT image shows the smooth, hyperattenuating focal thickening of the posterior retina of the left eye (arrowhead).
|
|
View larger version (153K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 5b. Retinocytoma in a 3-year-old boy with left strabismus and leukocoria. (a) Photograph of the sectioned gross specimen shows a homogeneous white, smooth, biconvex mass (arrowhead) in the posterior pole that abuts the retina. (b) Axial unenhanced CT image shows the smooth, hyperattenuating focal thickening of the posterior retina of the left eye (arrowhead).
|
|
View larger version (163K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 6. Retinoblastoma in an 11-month-old boy who was noted by a pediatrician to have leukocoria. Photomicrograph (original magnification, x200; H-E stain) shows sheets of discohesive basophilic cells with scant cytoplasm (*) and foci of calcification in areas of necrosis (arrowheads).
|
|
View larger version (150K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7a. Retinoblastoma in a 7-year-old girl who complained of blurry vision in her right eye. (a) Photomicrograph (original magnification, x400; H-E stain) shows Flexner-Wintersteiner rosettes with central lumina (straight arrows). Numerous mitotic figures are noted (arrowheads) as well as areas of necrosis (*). Also note the central focus of proliferative vessels with plump endothelial cells (curved arrow). (b) Photomicrograph (original magnification, x20; H-E stain) shows a homogeneous mass of tumor cells in the posterior globe (*) that invade the optic nerve (arrows). (c) Photomicrograph (original magnification, x100; H-E stain) shows basophilic neoplastic cells in the optic disc (*) crossing the lamina cribrosa (arrows) into the optic nerve (arrowheads). (d) Photograph of the sectioned gross specimen shows an irregular whitish mass (*) arising from the thickened retina (arrowhead). (e) Ultrasonographic (US) image obtained with a high-frequency linear transducer shows the heterogeneous, nodular mass (arrow) in the globe apposed to the retina and posterior to the lens (arrowhead). (f) US image shows posterior acoustic shadowing (arrow).
|
|
View larger version (166K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7b. Retinoblastoma in a 7-year-old girl who complained of blurry vision in her right eye. (a) Photomicrograph (original magnification, x400; H-E stain) shows Flexner-Wintersteiner rosettes with central lumina (straight arrows). Numerous mitotic figures are noted (arrowheads) as well as areas of necrosis (*). Also note the central focus of proliferative vessels with plump endothelial cells (curved arrow). (b) Photomicrograph (original magnification, x20; H-E stain) shows a homogeneous mass of tumor cells in the posterior globe (*) that invade the optic nerve (arrows). (c) Photomicrograph (original magnification, x100; H-E stain) shows basophilic neoplastic cells in the optic disc (*) crossing the lamina cribrosa (arrows) into the optic nerve (arrowheads). (d) Photograph of the sectioned gross specimen shows an irregular whitish mass (*) arising from the thickened retina (arrowhead). (e) Ultrasonographic (US) image obtained with a high-frequency linear transducer shows the heterogeneous, nodular mass (arrow) in the globe apposed to the retina and posterior to the lens (arrowhead). (f) US image shows posterior acoustic shadowing (arrow).
|
|
View larger version (179K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7c. Retinoblastoma in a 7-year-old girl who complained of blurry vision in her right eye. (a) Photomicrograph (original magnification, x400; H-E stain) shows Flexner-Wintersteiner rosettes with central lumina (straight arrows). Numerous mitotic figures are noted (arrowheads) as well as areas of necrosis (*). Also note the central focus of proliferative vessels with plump endothelial cells (curved arrow). (b) Photomicrograph (original magnification, x20; H-E stain) shows a homogeneous mass of tumor cells in the posterior globe (*) that invade the optic nerve (arrows). (c) Photomicrograph (original magnification, x100; H-E stain) shows basophilic neoplastic cells in the optic disc (*) crossing the lamina cribrosa (arrows) into the optic nerve (arrowheads). (d) Photograph of the sectioned gross specimen shows an irregular whitish mass (*) arising from the thickened retina (arrowhead). (e) Ultrasonographic (US) image obtained with a high-frequency linear transducer shows the heterogeneous, nodular mass (arrow) in the globe apposed to the retina and posterior to the lens (arrowhead). (f) US image shows posterior acoustic shadowing (arrow).
|
|
View larger version (102K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7d. Retinoblastoma in a 7-year-old girl who complained of blurry vision in her right eye. (a) Photomicrograph (original magnification, x400; H-E stain) shows Flexner-Wintersteiner rosettes with central lumina (straight arrows). Numerous mitotic figures are noted (arrowheads) as well as areas of necrosis (*). Also note the central focus of proliferative vessels with plump endothelial cells (curved arrow). (b) Photomicrograph (original magnification, x20; H-E stain) shows a homogeneous mass of tumor cells in the posterior globe (*) that invade the optic nerve (arrows). (c) Photomicrograph (original magnification, x100; H-E stain) shows basophilic neoplastic cells in the optic disc (*) crossing the lamina cribrosa (arrows) into the optic nerve (arrowheads). (d) Photograph of the sectioned gross specimen shows an irregular whitish mass (*) arising from the thickened retina (arrowhead). (e) Ultrasonographic (US) image obtained with a high-frequency linear transducer shows the heterogeneous, nodular mass (arrow) in the globe apposed to the retina and posterior to the lens (arrowhead). (f) US image shows posterior acoustic shadowing (arrow).
|
|
View larger version (124K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7e. Retinoblastoma in a 7-year-old girl who complained of blurry vision in her right eye. (a) Photomicrograph (original magnification, x400; H-E stain) shows Flexner-Wintersteiner rosettes with central lumina (straight arrows). Numerous mitotic figures are noted (arrowheads) as well as areas of necrosis (*). Also note the central focus of proliferative vessels with plump endothelial cells (curved arrow). (b) Photomicrograph (original magnification, x20; H-E stain) shows a homogeneous mass of tumor cells in the posterior globe (*) that invade the optic nerve (arrows). (c) Photomicrograph (original magnification, x100; H-E stain) shows basophilic neoplastic cells in the optic disc (*) crossing the lamina cribrosa (arrows) into the optic nerve (arrowheads). (d) Photograph of the sectioned gross specimen shows an irregular whitish mass (*) arising from the thickened retina (arrowhead). (e) Ultrasonographic (US) image obtained with a high-frequency linear transducer shows the heterogeneous, nodular mass (arrow) in the globe apposed to the retina and posterior to the lens (arrowhead). (f) US image shows posterior acoustic shadowing (arrow).
|
|
View larger version (103K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7f. Retinoblastoma in a 7-year-old girl who complained of blurry vision in her right eye. (a) Photomicrograph (original magnification, x400; H-E stain) shows Flexner-Wintersteiner rosettes with central lumina (straight arrows). Numerous mitotic figures are noted (arrowheads) as well as areas of necrosis (*). Also note the central focus of proliferative vessels with plump endothelial cells (curved arrow). (b) Photomicrograph (original magnification, x20; H-E stain) shows a homogeneous mass of tumor cells in the posterior globe (*) that invade the optic nerve (arrows). (c) Photomicrograph (original magnification, x100; H-E stain) shows basophilic neoplastic cells in the optic disc (*) crossing the lamina cribrosa (arrows) into the optic nerve (arrowheads). (d) Photograph of the sectioned gross specimen shows an irregular whitish mass (*) arising from the thickened retina (arrowhead). (e) Ultrasonographic (US) image obtained with a high-frequency linear transducer shows the heterogeneous, nodular mass (arrow) in the globe apposed to the retina and posterior to the lens (arrowhead). (f) US image shows posterior acoustic shadowing (arrow).
|
|
View larger version (147K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 8a. Trilateral retinoblastoma in a child of unknown age. (a) Axial CT image enhanced with intravenous contrast material shows bilateral hyperattenuating nodular masses containing dense foci of calcification (arrowheads). (b) Axial CT image enhanced with intravenous contrast material shows a large, round, intensely enhancing mass in the pineal region, which causes hydrocephalus.
|
|
View larger version (138K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 8b. Trilateral retinoblastoma in a child of unknown age. (a) Axial CT image enhanced with intravenous contrast material shows bilateral hyperattenuating nodular masses containing dense foci of calcification (arrowheads). (b) Axial CT image enhanced with intravenous contrast material shows a large, round, intensely enhancing mass in the pineal region, which causes hydrocephalus.
|
|
View larger version (142K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 9a. PHPV in a 3-month-old healthy term infant whose mother noted an abnormal left pupil. (a) Photograph (H-E stain) of the whole-mount specimen shows chronic total retinal detachment with leaves of the retina coapted (straight arrow). The subretinal space is filled with eosinophilic serous fluid (curved arrows). The peripheral thickened retina (arrowheads) is adherent to the posterior aspect of the cataractous lens (*). (b) Photomicrograph (original magnification, x20; H-E stain) obtained at a higher magnification shows the dysplastic retina (arrowheads) adherent to a condensation of primary vitreal mesenchymal tissue (*) apposed to the posterior surface of the cataractous lens (arrow). (c) Sagittal T1-weighted MR image of the left eye shows a triangular mass (arrow) abutting the posterior lens and abnormal low signal intensity in the lens. Note also the abnormal increased signal intensity in the subretinal fluid, an appearance possibly due to protein content. (d) Sagittal T1-weighted MR image of the normal right eye shows normal configuration and high signal intensity of the lens (arrow) relative to the vitreous.
|
|
View larger version (121K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 9b. PHPV in a 3-month-old healthy term infant whose mother noted an abnormal left pupil. (a) Photograph (H-E stain) of the whole-mount specimen shows chronic total retinal detachment with leaves of the retina coapted (straight arrow). The subretinal space is filled with eosinophilic serous fluid (curved arrows). The peripheral thickened retina (arrowheads) is adherent to the posterior aspect of the cataractous lens (*). (b) Photomicrograph (original magnification, x20; H-E stain) obtained at a higher magnification shows the dysplastic retina (arrowheads) adherent to a condensation of primary vitreal mesenchymal tissue (*) apposed to the posterior surface of the cataractous lens (arrow). (c) Sagittal T1-weighted MR image of the left eye shows a triangular mass (arrow) abutting the posterior lens and abnormal low signal intensity in the lens. Note also the abnormal increased signal intensity in the subretinal fluid, an appearance possibly due to protein content. (d) Sagittal T1-weighted MR image of the normal right eye shows normal configuration and high signal intensity of the lens (arrow) relative to the vitreous.
|
|
View larger version (117K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 9c. PHPV in a 3-month-old healthy term infant whose mother noted an abnormal left pupil. (a) Photograph (H-E stain) of the whole-mount specimen shows chronic total retinal detachment with leaves of the retina coapted (straight arrow). The subretinal space is filled with eosinophilic serous fluid (curved arrows). The peripheral thickened retina (arrowheads) is adherent to the posterior aspect of the cataractous lens (*). (b) Photomicrograph (original magnification, x20; H-E stain) obtained at a higher magnification shows the dysplastic retina (arrowheads) adherent to a condensation of primary vitreal mesenchymal tissue (*) apposed to the posterior surface of the cataractous lens (arrow). (c) Sagittal T1-weighted MR image of the left eye shows a triangular mass (arrow) abutting the posterior lens and abnormal low signal intensity in the lens. Note also the abnormal increased signal intensity in the subretinal fluid, an appearance possibly due to protein content. (d) Sagittal T1-weighted MR image of the normal right eye shows normal configuration and high signal intensity of the lens (arrow) relative to the vitreous.
|
|
View larger version (116K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 9d. PHPV in a 3-month-old healthy term infant whose mother noted an abnormal left pupil. (a) Photograph (H-E stain) of the whole-mount specimen shows chronic total retinal detachment with leaves of the retina coapted (straight arrow). The subretinal space is filled with eosinophilic serous fluid (curved arrows). The peripheral thickened retina (arrowheads) is adherent to the posterior aspect of the cataractous lens (*). (b) Photomicrograph (original magnification, x20; H-E stain) obtained at a higher magnification shows the dysplastic retina (arrowheads) adherent to a condensation of primary vitreal mesenchymal tissue (*) apposed to the posterior surface of the cataractous lens (arrow). (c) Sagittal T1-weighted MR image of the left eye shows a triangular mass (arrow) abutting the posterior lens and abnormal low signal intensity in the lens. Note also the abnormal increased signal intensity in the subretinal fluid, an appearance possibly due to protein content. (d) Sagittal T1-weighted MR image of the normal right eye shows normal configuration and high signal intensity of the lens (arrow) relative to the vitreous.
|
|
View larger version (157K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 10. PHPV in a 2-year-old boy with an abnormal left eye at examination by a pediatrician. Axial CT image obtained after administration of intravenous contrast material shows a vertical septum posterior to the left lens with anterior tenting of the posterior retina (arrowhead).
|
|
View larger version (130K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 11a. Coats disease in an 18-month-old boy with right leukocoria. (a) Photograph of the sectioned gross specimen shows massive retinal detachment with the leaves of the retina apposed (arrow). The vitreous body is obliterated between the leaves of the retina, and the subretinal space (*) is filled with material containing brightly reflecting cholesterol crystals. (b) Photomicrograph (original magnification, x40; H-E stain) shows telangiectatic vessels (arrowheads) in the thickened retina with eosinophilic subretinal serous fluid containing cholesterol clefts (straight arrows), which are elongated spaces left behind by cholesterol crystals dissolved in the fixation process. Curved arrow = lens. (c) Photomicrograph (original magnification, x200; H-E stain) obtained at a higher magnification shows dilated, thin-walled vessels (arrows) in the gliotic retina. Note the intraretinal lipoproteinaceous exudates (*) and the lipid-laden macrophages (arrowheads) in the subretinal serous fluid. (d) Axial unenhanced CT image shows diffuse increased attenuation without calcifications in the vitreous body of the right eye relative to that in the left eye. (e) Axial fat-saturated T1-weighted MR image, obtained after intravenous administration of gadolinium contrast material, shows the V configuration of severely detached leaves of the retina (arrows) with no enhancement of the subretinal space.
|
|
View larger version (141K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 11b. Coats disease in an 18-month-old boy with right leukocoria. (a) Photograph of the sectioned gross specimen shows massive retinal detachment with the leaves of the retina apposed (arrow). The vitreous body is obliterated between the leaves of the retina, and the subretinal space (*) is filled with material containing brightly reflecting cholesterol crystals. (b) Photomicrograph (original magnification, x40; H-E stain) shows telangiectatic vessels (arrowheads) in the thickened retina with eosinophilic subretinal serous fluid containing cholesterol clefts (straight arrows), which are elongated spaces left behind by cholesterol crystals dissolved in the fixation process. Curved arrow = lens. (c) Photomicrograph (original magnification, x200; H-E stain) obtained at a higher magnification shows dilated, thin-walled vessels (arrows) in the gliotic retina. Note the intraretinal lipoproteinaceous exudates (*) and the lipid-laden macrophages (arrowheads) in the subretinal serous fluid. (d) Axial unenhanced CT image shows diffuse increased attenuation without calcifications in the vitreous body of the right eye relative to that in the left eye. (e) Axial fat-saturated T1-weighted MR image, obtained after intravenous administration of gadolinium contrast material, shows the V configuration of severely detached leaves of the retina (arrows) with no enhancement of the subretinal space.
|
|
View larger version (134K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 11c. Coats disease in an 18-month-old boy with right leukocoria. (a) Photograph of the sectioned gross specimen shows massive retinal detachment with the leaves of the retina apposed (arrow). The vitreous body is obliterated between the leaves of the retina, and the subretinal space (*) is filled with material containing brightly reflecting cholesterol crystals. (b) Photomicrograph (original magnification, x40; H-E stain) shows telangiectatic vessels (arrowheads) in the thickened retina with eosinophilic subretinal serous fluid containing cholesterol clefts (straight arrows), which are elongated spaces left behind by cholesterol crystals dissolved in the fixation process. Curved arrow = lens. (c) Photomicrograph (original magnification, x200; H-E stain) obtained at a higher magnification shows dilated, thin-walled vessels (arrows) in the gliotic retina. Note the intraretinal lipoproteinaceous exudates (*) and the lipid-laden macrophages (arrowheads) in the subretinal serous fluid. (d) Axial unenhanced CT image shows diffuse increased attenuation without calcifications in the vitreous body of the right eye relative to that in the left eye. (e) Axial fat-saturated T1-weighted MR image, obtained after intravenous administration of gadolinium contrast material, shows the V configuration of severely detached leaves of the retina (arrows) with no enhancement of the subretinal space.
|
|
View larger version (143K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 11d. Coats disease in an 18-month-old boy with right leukocoria. (a) Photograph of the sectioned gross specimen shows massive retinal detachment with the leaves of the retina apposed (arrow). The vitreous body is obliterated between the leaves of the retina, and the subretinal space (*) is filled with material containing brightly reflecting cholesterol crystals. (b) Photomicrograph (original magnification, x40; H-E stain) shows telangiectatic vessels (arrowheads) in the thickened retina with eosinophilic subretinal serous fluid containing cholesterol clefts (straight arrows), which are elongated spaces left behind by cholesterol crystals dissolved in the fixation process. Curved arrow = lens. (c) Photomicrograph (original magnification, x200; H-E stain) obtained at a higher magnification shows dilated, thin-walled vessels (arrows) in the gliotic retina. Note the intraretinal lipoproteinaceous exudates (*) and the lipid-laden macrophages (arrowheads) in the subretinal serous fluid. (d) Axial unenhanced CT image shows diffuse increased attenuation without calcifications in the vitreous body of the right eye relative to that in the left eye. (e) Axial fat-saturated T1-weighted MR image, obtained after intravenous administration of gadolinium contrast material, shows the V configuration of severely detached leaves of the retina (arrows) with no enhancement of the subretinal space.
|
|
View larger version (146K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 11e. Coats disease in an 18-month-old boy with right leukocoria. (a) Photograph of the sectioned gross specimen shows massive retinal detachment with the leaves of the retina apposed (arrow). The vitreous body is obliterated between the leaves of the retina, and the subretinal space (*) is filled with material containing brightly reflecting cholesterol crystals. (b) Photomicrograph (original magnification, x40; H-E stain) shows telangiectatic vessels (arrowheads) in the thickened retina with eosinophilic subretinal serous fluid containing cholesterol clefts (straight arrows), which are elongated spaces left behind by cholesterol crystals dissolved in the fixation process. Curved arrow = lens. (c) Photomicrograph (original magnification, x200; H-E stain) obtained at a higher magnification shows dilated, thin-walled vessels (arrows) in the gliotic retina. Note the intraretinal lipoproteinaceous exudates (*) and the lipid-laden macrophages (arrowheads) in the subretinal serous fluid. (d) Axial unenhanced CT image shows diffuse increased attenuation without calcifications in the vitreous body of the right eye relative to that in the left eye. (e) Axial fat-saturated T1-weighted MR image, obtained after intravenous administration of gadolinium contrast material, shows the V configuration of severely detached leaves of the retina (arrows) with no enhancement of the subretinal space.
|
|
View larger version (77K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 12. Toxocara endophthalmitis in a 1-year-old girl. Axial unenhanced CT image shows a nonspecific mass of increased attenuation without calcification in the posterior left globe (arrowhead). (Case courtesy of Charles M. Glasier, MD, Arkansas Children’s Hospital, Little Rock.)
|
|
View larger version (143K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 13a. Malignant teratoid medulloepithelioma of the optic disc and retina in a 13-month-old girl with right exotropia and intermittent periocular redness. (a) Photomicrograph (original magnification, x400; H-E stain) shows irregular skeins of neoplastic cells arranged in the netlike or diktyomatous pattern (arrow) surrounded by basophilic fluid (*). Residual gliotic retina (R) is noted adjacent to the neoplasm. (b) Axial unenhanced CT image of the right globe shows a posterior hyperattenuating mass without calcification (arrowhead). At pathologic examination, the tumor arose from the posterior retina and optic disc.
|
|
View larger version (141K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 13b. Malignant teratoid medulloepithelioma of the optic disc and retina in a 13-month-old girl with right exotropia and intermittent periocular redness. (a) Photomicrograph (original magnification, x400; H-E stain) shows irregular skeins of neoplastic cells arranged in the netlike or diktyomatous pattern (arrow) surrounded by basophilic fluid (*). Residual gliotic retina (R) is noted adjacent to the neoplasm. (b) Axial unenhanced CT image of the right globe shows a posterior hyperattenuating mass without calcification (arrowhead). At pathologic examination, the tumor arose from the posterior retina and optic disc.
|
|
View larger version (93K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 14a. Pilocytic astrocytoma of the optic nerve in a 16-year-old girl who complained of progressive loss of vision in the right eye. (a) Photograph of the resected specimen shows fusiform enlargement of the optic nerve. (b) Contrast-enhanced fat-saturated T1-weighted MR image shows the fusiform enhancing mass (arrow) in the right orbital apex. (c) T1-weighted MR image shows enlargement of the isointense right optic nerve (arrow) relative to the normal left optic nerve.
|
|
View larger version (153K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 14b. Pilocytic astrocytoma of the optic nerve in a 16-year-old girl who complained of progressive loss of vision in the right eye. (a) Photograph of the resected specimen shows fusiform enlargement of the optic nerve. (b) Contrast-enhanced fat-saturated T1-weighted MR image shows the fusiform enhancing mass (arrow) in the right orbital apex. (c) T1-weighted MR image shows enlargement of the isointense right optic nerve (arrow) relative to the normal left optic nerve.
|
|
View larger version (120K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 14c. Pilocytic astrocytoma of the optic nerve in a 16-year-old girl who complained of progressive loss of vision in the right eye. (a) Photograph of the resected specimen shows fusiform enlargement of the optic nerve. (b) Contrast-enhanced fat-saturated T1-weighted MR image shows the fusiform enhancing mass (arrow) in the right orbital apex. (c) T1-weighted MR image shows enlargement of the isointense right optic nerve (arrow) relative to the normal left optic nerve.
|
|
View larger version (150K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 15a. Pilocytic astrocytoma of the optic pathway in a 5-year-old girl with right proptosis and headaches who was otherwise healthy. (a) Photomicrograph (original magnification, x20; H-E stain) shows a thick layer of neoplastic cells mingled with reactive leptomeningeal tissue (*) between the optic nerve (arrows) and the dura (arrowhead). (b) Photomicrograph (original magnification, x400; H-E stain) of an area of transition between compactly and loosely distributed neoplastic astrocytes shows piloid neoplastic cells with ellipsoid nuclei and long eosinophilic cytoplasmic processes (arrowheads). Note also the eosinophilic piloid Rosenthal fiber (arrow). (c) Photomicrograph (original magnification, x400; H-E stain) shows a curvilinear area of proliferative vessels (arrowheads) surrounding a cystic space (C) adjacent to an area of loosely arranged neoplastic astrocytes (*). (d) Axial T2-weighted MR image shows an intraconal hyperintense oblong mass (*) that causes proptosis on the right. (e) Sagittal T1-weighted MR image shows the bilobed mass of intermediate signal intensity (*) in the intraorbital, intracanalicular, and retrocanalicular portions of the optic nerve. (f) Sagittal fat-suppressed T1-weighted MR image, obtained in the same plane after intravenous administration of gadolinium contrast material, shows marked peripheral enhancement of the mass. The unenhancing central portion corresponded to cystic spaces seen at pathologic examination.
|
|
View larger version (141K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 15b. Pilocytic astrocytoma of the optic pathway in a 5-year-old girl with right proptosis and headaches who was otherwise healthy. (a) Photomicrograph (original magnification, x20; H-E stain) shows a thick layer of neoplastic cells mingled with reactive leptomeningeal tissue (*) between the optic nerve (arrows) and the dura (arrowhead). (b) Photomicrograph (original magnification, x400; H-E stain) of an area of transition between compactly and loosely distributed neoplastic astrocytes shows piloid neoplastic cells with ellipsoid nuclei and long eosinophilic cytoplasmic processes (arrowheads). Note also the eosinophilic piloid Rosenthal fiber (arrow). (c) Photomicrograph (original magnification, x400; H-E stain) shows a curvilinear area of proliferative vessels (arrowheads) surrounding a cystic space (C) adjacent to an area of loosely arranged neoplastic astrocytes (*). (d) Axial T2-weighted MR image shows an intraconal hyperintense oblong mass (*) that causes proptosis on the right. (e) Sagittal T1-weighted MR image shows the bilobed mass of intermediate signal intensity (*) in the intraorbital, intracanalicular, and retrocanalicular portions of the optic nerve. (f) Sagittal fat-suppressed T1-weighted MR image, obtained in the same plane after intravenous administration of gadolinium contrast material, shows marked peripheral enhancement of the mass. The unenhancing central portion corresponded to cystic spaces seen at pathologic examination.
|
|
View larger version (143K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 15c. Pilocytic astrocytoma of the optic pathway in a 5-year-old girl with right proptosis and headaches who was otherwise healthy. (a) Photomicrograph (original magnification, x20; H-E stain) shows a thick layer of neoplastic cells mingled with reactive leptomeningeal tissue (*) between the optic nerve (arrows) and the dura (arrowhead). (b) Photomicrograph (original magnification, x400; H-E stain) of an area of transition between compactly and loosely distributed neoplastic astrocytes shows piloid neoplastic cells with ellipsoid nuclei and long eosinophilic cytoplasmic processes (arrowheads). Note also the eosinophilic piloid Rosenthal fiber (arrow). (c) Photomicrograph (original magnification, x400; H-E stain) shows a curvilinear area of proliferative vessels (arrowheads) surrounding a cystic space (C) adjacent to an area of loosely arranged neoplastic astrocytes (*). (d) Axial T2-weighted MR image shows an intraconal hyperintense oblong mass (*) that causes proptosis on the right. (e) Sagittal T1-weighted MR image shows the bilobed mass of intermediate signal intensity (*) in the intraorbital, intracanalicular, and retrocanalicular portions of the optic nerve. (f) Sagittal fat-suppressed T1-weighted MR image, obtained in the same plane after intravenous administration of gadolinium contrast material, shows marked peripheral enhancement of the mass. The unenhancing central portion corresponded to cystic spaces seen at pathologic examination.
|
|
View larger version (131K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 15d. Pilocytic astrocytoma of the optic pathway in a 5-year-old girl with right proptosis and headaches who was otherwise healthy. (a) Photomicrograph (original magnification, x20; H-E stain) shows a thick layer of neoplastic cells mingled with reactive leptomeningeal tissue (*) between the optic nerve (arrows) and the dura (arrowhead). (b) Photomicrograph (original magnification, x400; H-E stain) of an area of transition between compactly and loosely distributed neoplastic astrocytes shows piloid neoplastic cells with ellipsoid nuclei and long eosinophilic cytoplasmic processes (arrowheads). Note also the eosinophilic piloid Rosenthal fiber (arrow). (c) Photomicrograph (original magnification, x400; H-E stain) shows a curvilinear area of proliferative vessels (arrowheads) surrounding a cystic space (C) adjacent to an area of loosely arranged neoplastic astrocytes (*). (d) Axial T2-weighted MR image shows an intraconal hyperintense oblong mass (*) that causes proptosis on the right. (e) Sagittal T1-weighted MR image shows the bilobed mass of intermediate signal intensity (*) in the intraorbital, intracanalicular, and retrocanalicular portions of the optic nerve. (f) Sagittal fat-suppressed T1-weighted MR image, obtained in the same plane after intravenous administration of gadolinium contrast material, shows marked peripheral enhancement of the mass. The unenhancing central portion corresponded to cystic spaces seen at pathologic examination.
|
|
View larger version (118K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 15e. Pilocytic astrocytoma of the optic pathway in a 5-year-old girl with right proptosis and headaches who was otherwise healthy. (a) Photomicrograph (original magnification, x20; H-E stain) shows a thick layer of neoplastic cells mingled with reactive leptomeningeal tissue (*) between the optic nerve (arrows) and the dura (arrowhead). (b) Photomicrograph (original magnification, x400; H-E stain) of an area of transition between compactly and loosely distributed neoplastic astrocytes shows piloid neoplastic cells with ellipsoid nuclei and long eosinophilic cytoplasmic processes (arrowheads). Note also the eosinophilic piloid Rosenthal fiber (arrow). (c) Photomicrograph (original magnification, x400; H-E stain) shows a curvilinear area of proliferative vessels (arrowheads) surrounding a cystic space (C) adjacent to an area of loosely arranged neoplastic astrocytes (*). (d) Axial T2-weighted MR image shows an intraconal hyperintense oblong mass (*) that causes proptosis on the right. (e) Sagittal T1-weighted MR image shows the bilobed mass of intermediate signal intensity (*) in the intraorbital, intracanalicular, and retrocanalicular portions of the optic nerve. (f) Sagittal fat-suppressed T1-weighted MR image, obtained in the same plane after intravenous administration of gadolinium contrast material, shows marked peripheral enhancement of the mass. The unenhancing central portion corresponded to cystic spaces seen at pathologic examination.
|
|
View larger version (124K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 15f. Pilocytic astrocytoma of the optic pathway in a 5-year-old girl with right proptosis and headaches who was otherwise healthy. (a) Photomicrograph (original magnification, x20; H-E stain) shows a thick layer of neoplastic cells mingled with reactive leptomeningeal tissue (*) between the optic nerve (arrows) and the dura (arrowhead). (b) Photomicrograph (original magnification, x400; H-E stain) of an area of transition between compactly and loosely distributed neoplastic astrocytes shows piloid neoplastic cells with ellipsoid nuclei and long eosinophilic cytoplasmic processes (arrowheads). Note also the eosinophilic piloid Rosenthal fiber (arrow). (c) Photomicrograph (original magnification, x400; H-E stain) shows a curvilinear area of proliferative vessels (arrowheads) surrounding a cystic space (C) adjacent to an area of loosely arranged neoplastic astrocytes (*). (d) Axial T2-weighted MR image shows an intraconal hyperintense oblong mass (*) that causes proptosis on the right. (e) Sagittal T1-weighted MR image shows the bilobed mass of intermediate signal intensity (*) in the intraorbital, intracanalicular, and retrocanalicular portions of the optic nerve. (f) Sagittal fat-suppressed T1-weighted MR image, obtained in the same plane after intravenous administration of gadolinium contrast material, shows marked peripheral enhancement of the mass. The unenhancing central portion corresponded to cystic spaces seen at pathologic examination.
|
|
View larger version (188K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 16. Optic pathway glioma in a 5-month-old girl with proptosis and afferent pupillary defect in the right eye. Axial CT image obtained after intravenous administration of contrast material shows an enhancing intraconal mass (arrowhead) that causes right proptosis; the mass extends along the optic nerve to involve the chiasm and right optic tract and radiations (arrow). Enhancing tumor surrounds the optic chiasm.
|
|
View larger version (147K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 17a. Bilateral optic pathway gliomas in a 2-year-old boy with NF1. (a) Axial T2-weighted MR image shows diffuse enlargement and tortuosity of both optic nerves (arrows). Also note the ill-defined foci of T2 prolongation in the pons (arrowhead), which are consistent with neurofibromatosis spots. (b) Coronal T2-weighted MR image shows T2 prolongation within the enlarged optic nerves (arrows).
|
|
View larger version (127K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 17b. Bilateral optic pathway gliomas in a 2-year-old boy with NF1. (a) Axial T2-weighted MR image shows diffuse enlargement and tortuosity of both optic nerves (arrows). Also note the ill-defined foci of T2 prolongation in the pons (arrowhead), which are consistent with neurofibromatosis spots. (b) Coronal T2-weighted MR image shows T2 prolongation within the enlarged optic nerves (arrows).
|
|
Copyright © 2007 by the Radiological Society of North America.
