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Research ArticleHead and Neck ImagingF
Open Access

Contrast-Enhancement of the Anterior Eye Segment in Patients with Retinoblastoma: Correlation between Clinical, MR Imaging, and Histopathologic Findings

P. de Graaf, P. van der Valk, A.C. Moll, S.M. Imhof, A.Y.N. Schouten-van Meeteren, D.L. Knol and J.A. Castelijns
American Journal of Neuroradiology February 2010, 31 (2) 237-245; DOI: https://doi.org/10.3174/ajnr.A1825
P. de Graaf
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P. van der Valk
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A.C. Moll
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S.M. Imhof
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A.Y.N. Schouten-van Meeteren
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D.L. Knol
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J.A. Castelijns
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  • Fig 1.
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    Fig 1.

    Transverse contrast-enhanced T1-weighted fat-suppressed spin-echo MR images of 3 different patients with unilateral retinoblastoma of the right eye show the grading of AES enhancement on a 3-point scale: normal AES enhancement (no perceptible enhancement within the AES) (A, arrow); moderate AES enhancement (less than choroidal enhancement) (B, arrow); and strong AES enhancement (equal to or greater than the intensity of choroidal enhancement) (C, arrow). All the unaffected (left) eyes show normal AES enhancement.

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    Fig 2.

    Transverse T2-weighted spin-echo MR images of 2 different patients with unilateral retinoblastoma of the left eye. A, A large intraocular retinoblastoma and a normal AC depth in comparison with the unaffected right eye. B, A large intraocular retinoblastoma and a decrease in AC depth (arrow) in comparison with the unaffected right eye.

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    Fig 3.

    Unilateral retinoblastoma in a 29-month-old boy (case 12). A, Transverse contrast-enhanced T1-weighted fat-suppressed spin-echo MR image shows a retinal detachment (arrow), with diffuse retinal thickening and enhancement due to retinoblastoma in the affected right eye. Compared with AES of the normal left eye, a strong abnormal enhancement is shown in the right eye. B, Histopathologic specimen of the iris, AC, and C, with details of the anterior iris surface (inset). The anatomic anterior surface of the iris (arrowheads) is covered by a prominent vascular membrane of newly formed capillaries (short arrow) (IA, Pe'er stage 3). Some vessels are present directly beneath the anatomic anterior iris surface (long arrow) (H&E, original magnification ×20 objective). C, IA at the anterior iris surface (arrow) is confirmed on additional CD31 staining (arrow) (×40 objective). D and E, VEGF expression in the iris is particularly present in the anterior part of the iris (arrow), and Flt-1 expression shows a widespread staining of the iris stroma (asterisk).

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    Fig 4.

    Unilateral retinoblastoma in a 6-month-old boy (case 13). A, Transverse contrast-enhanced T1-weighted fat-suppressed spin-echo MR image shows a large enhancing tumor mass in the left eye combined with a strong abnormal enhancement of the AES (arrow). B, Histopathologic specimen shows detail of the iris. Marked increase of blood vessels directly beneath the anatomic anterior iris surface (arrowheads) is present in this part of the iris (H&E, original magnification ×40 objective). C, CD31 staining shows a layer of fine capillaries (arrow) covering the anatomic anterior iris surface (IA, Pe'er stage 2) (original magnification ×40 objective).

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    Fig 5.

    Unilateral retinoblastoma in a 2-month-old boy (case 5). A, Transverse contrast-enhanced T1-weighted fat-suppressed spin-echo MR image shows a large enhancing tumor mass in the left eye. Symmetric normal enhancement of the AES of both eyes is shown. B, Histopathologic specimen shows details of the iris with a normal aspect of the anterior surface (H&E, original magnification ×40 objective). C, CD31 staining shows the absence of vessels outside the anterior iris surface (no IA, Pe'er stage 0) (original magnification ×40 objective).

Tables

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    Table 1:

    Distribution of contrast enhancement in the AES in relation to clinical findings in 42 eyes with retinoblastomaa

    VariableNormal (n = 13)Moderate (n = 15)Strong (n = 14)P Value
    Tumor recurrence1.00
        Yes0 (0)1 (7)0 (0)
        No13 (100)14 (93)14 (100)
    Intraocular pressure.61
        Unknown9 (69)7 (47)4 (29)
        Mean ± SD (mm Hg)11.8 ± 5.724.1 ± 9.619.0
        Range (mm Hg)7–2011–3610–37
    Vitreous seeding.17
        Yes3 (23)8 (57)7 (54)
        No10 (77)6 (43)6 (46)
    Subretinal seeding.31
        Yes6 (46)5 (36)3 (23)
        No7 (54)9 (64)10 (77)
    Anterior chamber seeding1.00
        Yes0 (0)1 (7)0 (0)
        No13 (100)14 (93)14 (100)
    Anterior chamber depth.07
        Shallow0 (0)3 (20)4 (29)
        Normal13 (100)12 (80)10 (71)
    Pseudohypopyon1.00
        Yes0 (0)1 (7)0 (0)
        No13 (100)14 (93)14 (100)
    Hyperemia.20
        Yes0 (0)4 (27)3 (21)
        No13 (100)11 (73)11 (79)
    IA.20
        Yes0 (0)0 (0)2 (14)
        No13 (100)15 (100)12 (86)
    • a Unless otherwise indicated, data are the number of eyes, and numbers in parentheses are percentages.

    • View popup
    Table 2:

    Distribution of contrast enhancement in the AES in relation to MR imaging findings in 42 eyes with retinoblastomaa

    VariableNormal (n = 13)Moderate (n = 15)Strong (n = 14)P Value
    Retinal detachment.17
        Total detachment2 (15)6 (40)6 (43)
        Some amount of detachment7 (54)4 (27)6 (43)
        No detachment4 (31)5 (33)2 (14)
    Anterior chamber depth.02
        Shallow1 (8)4 (27)(50)
        Normal12 (92)11 (73)7 (50)
    Tumor volumeb.002
        Mean ± SD (mm3)932.8 ± 561.31492.7 ± 617.41514.4 ± 388.7
        Range (mm3)221.5–1845.0523.6–2463.5910.5–2106.8
            Very small5 (38)1 (7)0 (0)
            Small5 (38)6 (40)5 (36)
            Medium3 (23)3 (20)7 (50)
            Large0 (0)5 (33)2 (14)
    • a Unless otherwise indicated, data are the number of eyes, and numbers in parentheses are percentages. Percentages may not add up to 100% because of rounding.

    • b Log-transformed.

    • View popup
    Table 3:

    Distribution of contrast enhancement in the AES in relation to histopathologic findings in 42 eyes with retinoblastomaa

    VariableNormal (n = 13)Moderate (n = 15)Strong (n = 14)P Value
    Tumor location.88
        Anterior to equator2 (15)1 (7)1 (7)
        Posterior to equator8 (62)8 (53)8 (57)
        Combination3 (23)6 (40)5 (36)
    Necrosis.56
        Mean ± SD24.6 ± 20.034.0 ± 21.220.7 ± 11.9
        Range5–605–7010–50
    Choroidal inflammation.02
        Yes5 (39)4 (27)0 (0)
        No8 (62)11 (73)14 (100)
    Choroidal invasion.17
        Massive0 (0)0 (0)0 (0)
        Minimal4 (31)4 (27)1 (7)
        No9 (69)11 (73)13 (93)
    Scleral invasion1.00
        Yes0 (0)0 (0)0 (0)
        No13 (100)15 (100)14 (100)
    AES1.00
        Yes0 (0)1 (7)0 (0)
        No13 (100)14 (93)14 (100)
    Optic nerve invasion<.0001
        Postlaminar0 (0)1 (7)2 (14)
        Prelaminar2 (15)5 (33)10 (71)
        No11 (85)9 (60)2 (14)
    IA.06
        Stage 30 (0)0 (0)1 (7)
        Stage 20 (0)3 (20)1 (7)
        Stage 11 (8)4 (27)4 (29)
        Stage 012 (92)8 (53)8 (57)
    Iris vessel count/5 HPF.18
        Mean ± SD75.8 ± 11.983.9 ± 14.683.2 ± 14.6
        Range56–9347–10266–124
    Iris surface-vessel count/5 HPF<.0001b
        Mean ± SD3.7 ± 2.89.5 ± 6.612.8 ± 9.5
        Range0–91–261–32
    • a Unless otherwise indicated, data are the number of eyes, and numbers in parentheses are percentages.

    • b Square-root transformed for statistical analysis because of skewed distribution.

    • View popup
    Table 4:

    Immunohistochemical findings in 14 eyes with retinoblastoma

    Case No./Sex/Age (mo)AESVEGFFlt-1CD31
    EnhancementIris SurfaceIris StromaIris SurfaceIris StromaaIAb
    1/M/2NormalStrongWeakWeakWeak1
    2/F/25NormalWeakNegativeNegativeNegative0
    3/M/4NormalStrongWeakWeakWeak0
    4/M/12NormalNegativeNegativeNegativeNegative0
    5/M/31NormalStrongWeakNegativeWeak0
    6/F/26ModerateWeakNegativeNegativeWeak0
    7/F/16ModerateStrongWeakStrongWeak3
    8/F/2ModerateStrongWeakNegativeWeak0
    9/M/2ModerateStrongStrongNegativeWeak0
    10/M/36StrongWeakNegativeNegativeWeak1
    11/F/20StrongNegativeNegativeNegativeWeak2
    12/M/29StrongWeakNegativeNegativeWeak3
    13/M/6StrongStrongWeakStrongStrong2
    14/M/3StrongStrongWeakNegativeWeak1
    • a Significant correlation between AES enhancement and the data in this column (r = .555; P = .04)

    • b Significant correlation between AES enhancement and the data in this column (r = .665; P = .009). IA according to the Pe'er classification.12

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American Journal of Neuroradiology: 31 (2)
American Journal of Neuroradiology
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P. de Graaf, P. van der Valk, A.C. Moll, S.M. Imhof, A.Y.N. Schouten-van Meeteren, D.L. Knol, J.A. Castelijns
Contrast-Enhancement of the Anterior Eye Segment in Patients with Retinoblastoma: Correlation between Clinical, MR Imaging, and Histopathologic Findings
American Journal of Neuroradiology Feb 2010, 31 (2) 237-245; DOI: 10.3174/ajnr.A1825

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Contrast-Enhancement of the Anterior Eye Segment in Patients with Retinoblastoma: Correlation between Clinical, MR Imaging, and Histopathologic Findings
P. de Graaf, P. van der Valk, A.C. Moll, S.M. Imhof, A.Y.N. Schouten-van Meeteren, D.L. Knol, J.A. Castelijns
American Journal of Neuroradiology Feb 2010, 31 (2) 237-245; DOI: 10.3174/ajnr.A1825
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