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Research ArticleHead and Neck Imaging
Open Access

Orbital Lymphoproliferative Disorders (OLPDs): Value of MR Imaging for Differentiating Orbital Lymphoma from Benign OPLDs

K. Haradome, H. Haradome, Y. Usui, S. Ueda, T.C. Kwee, K. Saito, K. Tokuuye, J. Matsubayashi, T. Nagao and H. Goto
American Journal of Neuroradiology October 2014, 35 (10) 1976-1982; DOI: https://doi.org/10.3174/ajnr.A3986
K. Haradome
aFrom the Departments of Ophthalmology (K.H., Y.U., S.U., H.G.)
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H. Haradome
dDepartment of Radiology (H.H.), Nihon University School of Medicine, Tokyo, Japan
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Y. Usui
aFrom the Departments of Ophthalmology (K.H., Y.U., S.U., H.G.)
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S. Ueda
aFrom the Departments of Ophthalmology (K.H., Y.U., S.U., H.G.)
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T.C. Kwee
eDepartment of Radiology (T.C.K.), University Medical Center Utrecht, Utrecht, the Netherlands.
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K. Saito
bRadiology (K.S., K.T.)
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K. Tokuuye
bRadiology (K.S., K.T.)
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J. Matsubayashi
cAnatomic Pathology (J.M., T.N.), Tokyo Medical University, Tokyo, Japan
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T. Nagao
cAnatomic Pathology (J.M., T.N.), Tokyo Medical University, Tokyo, Japan
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H. Goto
aFrom the Departments of Ophthalmology (K.H., Y.U., S.U., H.G.)
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  • Fig 1.
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    Fig 1.

    Orbital lymphoma (MALT lymphoma) in a 79-year-old woman. A, Transverse fat-saturated T2-weighted image; B, Transverse postcontrast fat-saturated T1-weighted image; C, Transverse DWI; D, ADC map; and E, Hematoxylin and eosin (HE)-stained biopsied specimen of the orbital tumor at low power field. An infiltrative mass involves the left lacrimal gland and extraocular muscles, appears isointense compared with the brain cortex on a fat-saturated T2-weighted MR image (A), and shows homogeneous contrast enhancement, similar to that of the right extraocular muscle (arrow) (B). The mass appears as strongly hyperintense on DWI (C) and hypointense on the ADC map (D). Notably, noninvolved extraocular muscles do not show hyperintensity similar to that of the mass on DWI (C). Lesion ADC and CER were 0.56 × 10−3 mm2/s and 1.69, respectively. HE-stained biopsied specimen (E) demonstrated numerous small-to-medium-sized atypical lymphocytes around reactive lymph follicles, with greater high cellular attenuation, however, interstitial fibrosis or edematous change was not prominent.

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    Fig 2.

    IgG4-related ophthalmic disease in a 67-year-old woman. A, Transverse fat-saturated T2-weighted image; B, transverse postcontrast fat-saturated T1-weighted image; C, transverse DWI; D, ADC map; and E, Hematoxylin and eosin (HE)-stained biopsied specimen of the lacrimal gland at low power field. The mass-like enlarged lacrimal glands are isointense compared with the brain cortex on the fat-saturated T2-weighted image (A) and show homogeneous contrast enhancement, similar to that of the extraocular muscles (B). The lesions appear mildly hyperintense on DWI (C) and slightly hypointense on the ADC map (D). Lesion ADC and CER were 0.94/0.75 (right/left) ×10−3 mm2/s and 2.05/2.07 (right/left), respectively. HE-stained biopsied specimen (E) showed a large germinal center with accompanying lymphoplasmacytic infiltration and abundant interstitial fibrosis with edematous changes. On immunochemical stained section analysis (not shown), many IgG4-positive plasma cell (>40%) were identified, and this is compatible with IgG4-related ophthalmic disease.

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    Fig 3.

    Orbital lymphoma (mantle cell lymphoma) in a 71-year-old man. A, Transverse fat-saturated T2-weighted image; B, transverse fat-saturated postcontrast T1-weighted image; C, transverse DWI; and D, ADC map. The lesions involve the bilateral lacrimal glands, appear isointense compared with the brain cortex on fat-saturated T2-weighted image (A), and show homogeneous contrast enhancement, similar that of the extraocular muscles (B). The lesions are strongly hyperintense on the DWI (C) and hypointense on the ADC map (D). Lesion ADC and CER were 0.47/0.48 (right/left) ×10−3 mm2/s and 1.49/1.46 (right/left), respectively. The low ADC and CER values could suggest orbital lymphoma though the imaging features are similar to that of IgG4-related ophthalmic disease (Fig 2).

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    Fig 4.

    Reactive lymphoid hyperplasia in a 65-year-old man. A, Coronal fat-saturated T2-weighted image. A number of vessel signal voids are observed in the bilaterally enlarged lacrimal glands on T2-weighted image (“flow void sign”) (arrows).

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    Fig 5.

    ADCs and CERs of orbital lymphoma and benign OPLDs. A and B, Scatterplot and box-and-whisker plot show the distribution of ADCs and CERs, and each mean value in the orbital lymphoma and benign OLPDs lesions. The mean ADC and CER of orbital lymphomas are significantly lower than those of benign OLPDs (P < .01). The standard deviation of the ADC of orbital lymphomas was lower (±0.051) compared with that of benign OLPDs (±0.246), and ADCs of only 2 benign OLPDs (encircled) overlapped with those of orbital lymphomas in the ADC range.

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    Table 1:

    Patient characteristics and pathologic findings of OLPD cases

    Orbital Lymphoma (n = 29)Benign OLPDs (n = 18)P Value
    Age72.2 ± 11.3 (47–88)57.6 ± 14.7 (27–80)P = .001a
    SexMale 18, Female 11Male 10, Female 8P = .763
    Histologic subtypesMALT lymphoma 21 (72)IgG4-related ophthalmic disease 14 (78)
    DLBCL 4 (14)RLH 4 (22)
    Follicular lymphoma 3 (10)
    Mantle cell lymphoma 1 (3)
    LateralityBilateral 12 (41)Bilateral 15 (83)P = .006a
    Unilateral 17 (59)Unilateral 3 (17)
    • Note:—Data in parentheses are ranges for age and percentages for histologic subtypes and laterality. DLBCL indicates diffuse large B-cell lymphoma; RLH, reactive lymphoid hyperplasia.

    • ↵a There were significant differences in age and laterality between the 2 groups.

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    Table 2:

    MR imaging characteristics

    Orbital Lymphoma (n = 29)Benign OLPDs (n = 18)P Value
    ShapeWell-defined 7 (24)Well-defined 10 (56)P = .0006a
    Ill-defined 21 (72)Ill-defined 3 (17)
    Lobulated 1 (3)Lobulated 5 (17)
    Signal intensity
        T1WIIso 28 (97)Iso 18 (100)P = .2548
    Low 1 (3)
        T2WIIso 27 (93)Iso 16 (89)P = .6147
    Low 2 (7)Low 2 (11)
        DWIHigh 17 (100)High 14 (93)P = .2794
    Iso 1 (7)
    Homogeneity and degree of contrast enhancementHomogeneous 23 (85)Homogeneous 15 (100)P = .2787
    Inhomogeneous 4 (15)
    High 2 (13)P = .0519
    Iso 27 (100)Iso 13 (87)
    Presence of “flow void sign”17 (59)17 (94)P = .0084a
    Findings suggestive of sinusitis8 (28)16 (89)P < .001a
    • Note:—Data in parentheses are percentages.

    • ↵a There were significant differences in shape of the lesions, presence of the “flow void sign,” and findings suggestive of sinusitis between the 2 groups.

    • View popup
    Table 3:

    ADCs of each group of OLPDs

    Mean ADC ± SDADC rangeP Value
    Orbital lymphoma (n = 17)0.54 ± 0.050.44–0.64P < .001a
    MALT lymphoma (n = 12)0.47–0.64
    DLBCL (n = 2)0.44–0.56
    Follicular lymphoma (n = 2)0.53–0.57
    Mantle cell lymphoma (n = 1)0.47
    Benign OLPDs (n = 15)0.81 ± 0.180.58–1.24
    IgG4-related ophthalmic disease (n = 12)0.58–1.24
    RLH (n = 3)0.73–0.85
    • Note:—There were significant differences in ADC between the 2 groups. SD indicates standard deviation; DLBCL diffuse large B-cell lymphoma; RLH, reactive lymphoid hyperplasia.

    • View popup
    Table 4:

    CERs of each group of OLPDs

    Mean CER ± SDCER RangeP Value
    Orbital lymphoma (n = 27)1.70 ± 0.251.27–2.24P = .0096a
    MALT lymphoma (n = 20)1.27–2.24
    DLBCL (n = 3)1.36–1.71
    Follicular lymphoma (n = 3)1.76–1.94
    Mantle cell lymphoma (n = 1)1.55
    Benign OLPDs (n = 15)2.07 ± 0.461.27–3.03
    IgG4-related ophthalmic disease (n = 11)1.27–3.03
    RLH (n = 4)1.66–2.14
    • ↵a There were significant differences in CER between the 2 groups. SD indicates standard deviation; DLBCL, diffuse large B-cell lymphoma; RLH, reactive lymphoid hyperplasia.

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K. Haradome, H. Haradome, Y. Usui, S. Ueda, T.C. Kwee, K. Saito, K. Tokuuye, J. Matsubayashi, T. Nagao, H. Goto
Orbital Lymphoproliferative Disorders (OLPDs): Value of MR Imaging for Differentiating Orbital Lymphoma from Benign OPLDs
American Journal of Neuroradiology Oct 2014, 35 (10) 1976-1982; DOI: 10.3174/ajnr.A3986

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Orbital Lymphoproliferative Disorders (OLPDs): Value of MR Imaging for Differentiating Orbital Lymphoma from Benign OPLDs
K. Haradome, H. Haradome, Y. Usui, S. Ueda, T.C. Kwee, K. Saito, K. Tokuuye, J. Matsubayashi, T. Nagao, H. Goto
American Journal of Neuroradiology Oct 2014, 35 (10) 1976-1982; DOI: 10.3174/ajnr.A3986
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