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Retinoblastoma: MR Imaging Parameters in Detection of Tumor Extent¹

¹ From the Departments of Radiology (P.d.G., F.B., J.A.C.), Ophthalmology (A.C.M., S.M.I.), Epidemiology and Statistics (D.L.K.), and Pathology (P.v.d.V.), VU University Medical Center, PO Box 7057, 1007 MB Amsterdam, the Netherlands. Received August 15, 2003; revision requested October 31; final revision received April 20, 2004; accepted June 17. Address correspondence to P.d.G. (e-mail: p.degraaf@vumc.nl).

View larger version (152K): [in a new window]   Figure 1. Bilateral retinoblastoma in 2-year-old boy. Transverse T1-weighted gadolinium-enhanced MR image (370/14) of left eye shows inhomogeneous enhancement pattern and local thickening of the choroid, adjacent to the tumor, and these findings were suspicious for extensive tumor invasion (arrows). Histopathologic examination results confirmed these findings.

View larger version (130K): [in a new window]   Figure 2a. Retinoblastoma in 18-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted MR image (500/15) of right eye shows retinoblastoma and choroid with similar SI. Choroidal invasion could not be excluded with MR imaging, because tumor and choroid showed similar SI. Notice asymmetric enhancement of the iris (arrow), which was clinically confirmed by the presence of rubeosis iridis. (b) Histopathologic examination did not show invasion of the choroid (C) by retinoblastoma (R). Choroid is not affected, because retinal pigment epithelium layer (arrowheads) is intact. (Hematoxylin-eosin stain; original magnification, x20 objective.)

View larger version (145K): [in a new window]   Figure 2b. Retinoblastoma in 18-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted MR image (500/15) of right eye shows retinoblastoma and choroid with similar SI. Choroidal invasion could not be excluded with MR imaging, because tumor and choroid showed similar SI. Notice asymmetric enhancement of the iris (arrow), which was clinically confirmed by the presence of rubeosis iridis. (b) Histopathologic examination did not show invasion of the choroid (C) by retinoblastoma (R). Choroid is not affected, because retinal pigment epithelium layer (arrowheads) is intact. (Hematoxylin-eosin stain; original magnification, x20 objective.)

View larger version (133K): [in a new window]   Figure 3a. Retinoblastoma in 3-year-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (575/15) of right eye shows tumor mass (arrowheads) surrounded by hyperintense subacute subretinal bleeding. Notice local thickening of choroid (black arrow) lateral to the optic nerve caused by massive tumor invasion. Increased SI (white arrow) posterior to the eyeball is present and was suspicious for postlaminar tumor invasion. (b) Transverse T2-weighted MR image (2200/120) shows characteristic hypointense retinoblastoma surrounded by hyperintense subretinal fluid. (c) Histopathologic specimen shows massive invasion of retinoblastoma (R) into the choroid (C). Sclera (S) is not affected. (Hematoxylin-eosin; original magnification, x10 objective.) (d) Histopathologic specimen shows same findings as in c and postlaminar optic nerve (O) invasion (arrow). (Hematoxylin-eosin; original magnification, x20 objective.)

View larger version (120K): [in a new window]   Figure 3b. Retinoblastoma in 3-year-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (575/15) of right eye shows tumor mass (arrowheads) surrounded by hyperintense subacute subretinal bleeding. Notice local thickening of choroid (black arrow) lateral to the optic nerve caused by massive tumor invasion. Increased SI (white arrow) posterior to the eyeball is present and was suspicious for postlaminar tumor invasion. (b) Transverse T2-weighted MR image (2200/120) shows characteristic hypointense retinoblastoma surrounded by hyperintense subretinal fluid. (c) Histopathologic specimen shows massive invasion of retinoblastoma (R) into the choroid (C). Sclera (S) is not affected. (Hematoxylin-eosin; original magnification, x10 objective.) (d) Histopathologic specimen shows same findings as in c and postlaminar optic nerve (O) invasion (arrow). (Hematoxylin-eosin; original magnification, x20 objective.)

View larger version (137K): [in a new window]   Figure 3c. Retinoblastoma in 3-year-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (575/15) of right eye shows tumor mass (arrowheads) surrounded by hyperintense subacute subretinal bleeding. Notice local thickening of choroid (black arrow) lateral to the optic nerve caused by massive tumor invasion. Increased SI (white arrow) posterior to the eyeball is present and was suspicious for postlaminar tumor invasion. (b) Transverse T2-weighted MR image (2200/120) shows characteristic hypointense retinoblastoma surrounded by hyperintense subretinal fluid. (c) Histopathologic specimen shows massive invasion of retinoblastoma (R) into the choroid (C). Sclera (S) is not affected. (Hematoxylin-eosin; original magnification, x10 objective.) (d) Histopathologic specimen shows same findings as in c and postlaminar optic nerve (O) invasion (arrow). (Hematoxylin-eosin; original magnification, x20 objective.)

View larger version (137K): [in a new window]   Figure 3d. Retinoblastoma in 3-year-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (575/15) of right eye shows tumor mass (arrowheads) surrounded by hyperintense subacute subretinal bleeding. Notice local thickening of choroid (black arrow) lateral to the optic nerve caused by massive tumor invasion. Increased SI (white arrow) posterior to the eyeball is present and was suspicious for postlaminar tumor invasion. (b) Transverse T2-weighted MR image (2200/120) shows characteristic hypointense retinoblastoma surrounded by hyperintense subretinal fluid. (c) Histopathologic specimen shows massive invasion of retinoblastoma (R) into the choroid (C). Sclera (S) is not affected. (Hematoxylin-eosin; original magnification, x10 objective.) (d) Histopathologic specimen shows same findings as in c and postlaminar optic nerve (O) invasion (arrow). (Hematoxylin-eosin; original magnification, x20 objective.)

View larger version (130K): [in a new window]   Figure 4a. Bilateral retinoblastoma in 10-month-old boy. (a) Transverse T1-weighted MR image (480/15) of left eye obtained after gadolinium-based contrast agent administration shows hyperintense enhancing ring, which appears to be a diffuse thickened choroid, probably related to inflammation secondary to an intraocular tumor (arrows). (b) Microscopic specimen shows a detail of optic nerve (O), choroid (C), and sclera (S). Inflammatory cells, present in choroid, caused MR image enhancement. (Hematoxylin-eosin stain; original magnification, x20 objective.)

View larger version (125K): [in a new window]   Figure 4b. Bilateral retinoblastoma in 10-month-old boy. (a) Transverse T1-weighted MR image (480/15) of left eye obtained after gadolinium-based contrast agent administration shows hyperintense enhancing ring, which appears to be a diffuse thickened choroid, probably related to inflammation secondary to an intraocular tumor (arrows). (b) Microscopic specimen shows a detail of optic nerve (O), choroid (C), and sclera (S). Inflammatory cells, present in choroid, caused MR image enhancement. (Hematoxylin-eosin stain; original magnification, x20 objective.)

View larger version (132K): [in a new window]   Figure 5a. Bilateral retinoblastoma in 2-month-old girl. (a) Transverse T1-weighted fat-suppressed MR image (541/15) of right eye obtained after administration of gadolinium-based contrast agent shows strong enhancement and diffuse thickening of the choroid, probably related to choroidal inflammation. This enhancement extends into the optic nerve (arrow) and was suspicious for postlaminar tumor invasion. (b) Microscopic specimen shows retinoblastoma (R), with massive necrosis, sclera (S), and inflammation of choroid (C). A granuloma (arrow) was detected in the optic nerve head and was responsible for contrast enhancement pattern on MR image. No tumor invasion into optic nerve (O), choroid, or sclera was present. (Hematoxylin-eosin stain; original magnification, x5 objective.)

View larger version (129K): [in a new window]   Figure 5b. Bilateral retinoblastoma in 2-month-old girl. (a) Transverse T1-weighted fat-suppressed MR image (541/15) of right eye obtained after administration of gadolinium-based contrast agent shows strong enhancement and diffuse thickening of the choroid, probably related to choroidal inflammation. This enhancement extends into the optic nerve (arrow) and was suspicious for postlaminar tumor invasion. (b) Microscopic specimen shows retinoblastoma (R), with massive necrosis, sclera (S), and inflammation of choroid (C). A granuloma (arrow) was detected in the optic nerve head and was responsible for contrast enhancement pattern on MR image. No tumor invasion into optic nerve (O), choroid, or sclera was present. (Hematoxylin-eosin stain; original magnification, x5 objective.)

View larger version (129K): [in a new window]   Figure 6a. Unilateral retinoblastoma in 20-month-old girl. (a) Transverse T1-weighted MR image (575/15) of right eye obtained after gadolinium-based contrast agent administration shows large tumor mass with inhomogeneous enhancement. Tumor invasion of the ciliary body (arrows) was suspected but was a false-positive finding, probably because of insufficient spatial resolution. The iris manifests as a hyperintense band anterior to the lens as a result of rubeosis iridis detected with ophthalmoscopic examination. (b) Histopathologic specimen shows a detail of the ciliary body (CB) (arrows) without tumor infiltration. Tumor (R) is adjacent to the ciliary body, anterior chamber, and iris (I). (Hematoxylin-eosin stain; original magnification, x10 objective.)

View larger version (134K): [in a new window]   Figure 6b. Unilateral retinoblastoma in 20-month-old girl. (a) Transverse T1-weighted MR image (575/15) of right eye obtained after gadolinium-based contrast agent administration shows large tumor mass with inhomogeneous enhancement. Tumor invasion of the ciliary body (arrows) was suspected but was a false-positive finding, probably because of insufficient spatial resolution. The iris manifests as a hyperintense band anterior to the lens as a result of rubeosis iridis detected with ophthalmoscopic examination. (b) Histopathologic specimen shows a detail of the ciliary body (CB) (arrows) without tumor infiltration. Tumor (R) is adjacent to the ciliary body, anterior chamber, and iris (I). (Hematoxylin-eosin stain; original magnification, x10 objective.)

View larger version (99K): [in a new window]   Figure 7a. Unilateral retinoblastoma in 12-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (475/14) shows an enhancing tumor mass (arrowheads) of right eye. Notice extreme enhancement of the iris (arrow) compared with appearance of left eye. Clinical examination revealed rubeosis iridis of the right eye. (b) Histopathologic specimen shows a detail of the anterior chamber (AC), cornea (CO), and lateral part of the iris (I). Marked increase of blood vessels is present in the iris (arrowheads). (Hematoxylin-eosin stain; original magnification, x20 objective.)

View larger version (72K): [in a new window]   Figure 7b. Unilateral retinoblastoma in 12-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (475/14) shows an enhancing tumor mass (arrowheads) of right eye. Notice extreme enhancement of the iris (arrow) compared with appearance of left eye. Clinical examination revealed rubeosis iridis of the right eye. (b) Histopathologic specimen shows a detail of the anterior chamber (AC), cornea (CO), and lateral part of the iris (I). Marked increase of blood vessels is present in the iris (arrowheads). (Hematoxylin-eosin stain; original magnification, x20 objective.)

View larger version (145K): [in a new window]   Figure 8a. Unilateral retinoblastoma in 12-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted MR image (475/14) of right eye shows prelaminar optic nerve invasion (arrow) as an interruption of linear enhancement pattern of the choroidoretinal complex at the optic disc. (This finding was rated as true-positive.) (b) Histopathologic specimen shows retinoblastoma (R) that infiltrated optic nerve (O) up to lamina cribrosa (LC), which indicated prelaminar invasion. (Hematoxylin-eosin stain; original magnification, x5 objective.)

View larger version (153K): [in a new window]   Figure 8b. Unilateral retinoblastoma in 12-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted MR image (475/14) of right eye shows prelaminar optic nerve invasion (arrow) as an interruption of linear enhancement pattern of the choroidoretinal complex at the optic disc. (This finding was rated as true-positive.) (b) Histopathologic specimen shows retinoblastoma (R) that infiltrated optic nerve (O) up to lamina cribrosa (LC), which indicated prelaminar invasion. (Hematoxylin-eosin stain; original magnification, x5 objective.)

View larger version (141K): [in a new window]   Figure 9a. Unilateral retinoblastoma in 20-month-old girl. (a) Transverse gadolinium-enhanced T1-weighted MR image (575/15) of right eye shows large tumor mass associated with prelaminar optic nerve invasion (arrow), which was not suspected. Notice absence of bulk of tumor mass near the optic disc. (b) Histopathologic specimen depicts optic disc (O) invaded by retinoblastoma (R). (Hematoxylin-eosin stain; original magnification, x10 objective.)

View larger version (143K): [in a new window]   Figure 9b. Unilateral retinoblastoma in 20-month-old girl. (a) Transverse gadolinium-enhanced T1-weighted MR image (575/15) of right eye shows large tumor mass associated with prelaminar optic nerve invasion (arrow), which was not suspected. Notice absence of bulk of tumor mass near the optic disc. (b) Histopathologic specimen depicts optic disc (O) invaded by retinoblastoma (R). (Hematoxylin-eosin stain; original magnification, x10 objective.)

View larger version (128K): [in a new window]   Figure 10a. Bilateral retinoblastoma in 15-month-old girl. (a) Transverse T1-weighted MR image (375/14) of left eye obtained after gadolinium-based contrast agent administration shows retinoblastoma at posterior part of the eyeball with normal thickness of underlying optic nerve disc and linear enhancement pattern of choroidoretinal complex (arrow) and choroid. Both prelaminar and postlaminar tumor invasion of the optic nerve were not suspected. (b) Histopathologic specimen of optic nerve (O) revealed postlaminar tumor (R) invasion (arrow). (Hematoxylin-eosin stain; original magnification, x10 objective.)

View larger version (131K): [in a new window]   Figure 10b. Bilateral retinoblastoma in 15-month-old girl. (a) Transverse T1-weighted MR image (375/14) of left eye obtained after gadolinium-based contrast agent administration shows retinoblastoma at posterior part of the eyeball with normal thickness of underlying optic nerve disc and linear enhancement pattern of choroidoretinal complex (arrow) and choroid. Both prelaminar and postlaminar tumor invasion of the optic nerve were not suspected. (b) Histopathologic specimen of optic nerve (O) revealed postlaminar tumor (R) invasion (arrow). (Hematoxylin-eosin stain; original magnification, x10 objective.)

View larger version (135K): [in a new window]   Figure 11a. Unilateral retinoblastoma in 26-month-old girl. (a) Transverse gadolinium-enhanced T1-weighted MR image (575/15) of left eye. Normal thickness of the optic disc is seen with linear enhancement pattern (arrow), a finding that was not suspicious for invasion of this structure. Enhancement of choroid (arrowheads) adjacent to the tumor does not indicate tumor infiltration. (b) Macroscopic histopathologic specimen shows retinoblastoma (R) and confirms absence of optic nerve (O) and choroidal (c) invasion. Retinal detachment (*) is an artifact caused by histopathologic preparation. (Hematoxylin-eosin stain; original magnification, x3.5 objective.)

View larger version (138K): [in a new window]   Figure 11b. Unilateral retinoblastoma in 26-month-old girl. (a) Transverse gadolinium-enhanced T1-weighted MR image (575/15) of left eye. Normal thickness of the optic disc is seen with linear enhancement pattern (arrow), a finding that was not suspicious for invasion of this structure. Enhancement of choroid (arrowheads) adjacent to the tumor does not indicate tumor infiltration. (b) Macroscopic histopathologic specimen shows retinoblastoma (R) and confirms absence of optic nerve (O) and choroidal (c) invasion. Retinal detachment (*) is an artifact caused by histopathologic preparation. (Hematoxylin-eosin stain; original magnification, x3.5 objective.)

View larger version (159K): [in a new window]   Figure 12. Unilateral retinoblastoma in 17-month-old girl. Transverse gadolinium-enhanced T1-weighted MR image (575/15) of left eye. Temporal part of the eye shows enhancing retinoblastoma (hyperintense signal), combined with subretinal hemorrhage (temporal and nasal). Choroidoretinal complex shows linear enhancement pattern (arrowhead), which indicates absence of prelaminar invasion. Normal thickness of optic disc is seen. Histopathologic examination results confirmed these findings.

View larger version (94K): [in a new window]   Figure 13a. Unilateral retinoblastoma in 14-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (575/15) of eyes. Contrast between tumor and vitreous is diminished because of subretinal blood. Increased SI of the postlaminar optic nerve in continuity with tumor was suspicious for postlaminar tumor invasion (long arrow). Notice shallowness of anterior chamber combined with SI increase of the iris (short arrow). Rubeosis iridis was detected with ophthalmoscopic examination. Shallowness is probably related to presence of neovascular glaucoma combined with elevated intraocular pressure, which is an indication for enucleation without delay. (b) Transverse T2-weighted MR image (2200/120) shows total retinal detachment (black arrow) with subretinal hypointense tumor mass (white arrow). Characteristic blood-fluid level (arrowheads) indicates acute subretinal hemorrhage. (c) Histopathologic specimen shows retinoblastoma (R) with postlaminar optic nerve invasion, detachment of retina (arrowheads), and shallow anterior chamber (AC). Choroid (C) and sclera (S) were not invaded by tumor. (Hematoxylin-eosin stain; original magnification, x3.5 objective.) Optic nerve (O) is also involved. Inset: Detail of optic nerve shows postlaminar tumor invasion (arrows). (Original magnification, x10 objective.)

View larger version (95K): [in a new window]   Figure 13b. Unilateral retinoblastoma in 14-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (575/15) of eyes. Contrast between tumor and vitreous is diminished because of subretinal blood. Increased SI of the postlaminar optic nerve in continuity with tumor was suspicious for postlaminar tumor invasion (long arrow). Notice shallowness of anterior chamber combined with SI increase of the iris (short arrow). Rubeosis iridis was detected with ophthalmoscopic examination. Shallowness is probably related to presence of neovascular glaucoma combined with elevated intraocular pressure, which is an indication for enucleation without delay. (b) Transverse T2-weighted MR image (2200/120) shows total retinal detachment (black arrow) with subretinal hypointense tumor mass (white arrow). Characteristic blood-fluid level (arrowheads) indicates acute subretinal hemorrhage. (c) Histopathologic specimen shows retinoblastoma (R) with postlaminar optic nerve invasion, detachment of retina (arrowheads), and shallow anterior chamber (AC). Choroid (C) and sclera (S) were not invaded by tumor. (Hematoxylin-eosin stain; original magnification, x3.5 objective.) Optic nerve (O) is also involved. Inset: Detail of optic nerve shows postlaminar tumor invasion (arrows). (Original magnification, x10 objective.)

View larger version (149K): [in a new window]   Figure 13c. Unilateral retinoblastoma in 14-month-old boy. (a) Transverse gadolinium-enhanced T1-weighted fat-suppressed MR image (575/15) of eyes. Contrast between tumor and vitreous is diminished because of subretinal blood. Increased SI of the postlaminar optic nerve in continuity with tumor was suspicious for postlaminar tumor invasion (long arrow). Notice shallowness of anterior chamber combined with SI increase of the iris (short arrow). Rubeosis iridis was detected with ophthalmoscopic examination. Shallowness is probably related to presence of neovascular glaucoma combined with elevated intraocular pressure, which is an indication for enucleation without delay. (b) Transverse T2-weighted MR image (2200/120) shows total retinal detachment (black arrow) with subretinal hypointense tumor mass (white arrow). Characteristic blood-fluid level (arrowheads) indicates acute subretinal hemorrhage. (c) Histopathologic specimen shows retinoblastoma (R) with postlaminar optic nerve invasion, detachment of retina (arrowheads), and shallow anterior chamber (AC). Choroid (C) and sclera (S) were not invaded by tumor. (Hematoxylin-eosin stain; original magnification, x3.5 objective.) Optic nerve (O) is also involved. Inset: Detail of optic nerve shows postlaminar tumor invasion (arrows). (Original magnification, x10 objective.)