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      Computed tomography and magnetic resonance imaging findings of intraorbital granular cell tumor (Abrikossoff’s tumor): a case report

      case-report

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          Abstract

          Background

          Granular cell tumors are rare neoplasms which can occur in any part of the body. Granular cell tumors of the orbit account for only 3 % of all granular cell tumor cases. Computed tomography and magnetic resonance imaging of the orbit have proven useful for diagnosing orbital tumors. However, the rarity of intraorbital granular cell tumors poses a significant diagnostic challenge for both clinicians and radiologists.

          Case presentation

          We report a case of a 37-year-old Chinese woman with a rare intraocular granular cell tumor of her right eye presenting with diplopia, proptosis, and restriction of ocular movement. Preoperative orbital computed tomography and magnetic resonance imaging with contrast enhancement revealed an enhancing solid, ovoid, well-demarcated, retrobulbar nodule. In addition, magnetic resonance imaging features included an intraorbital tumor which was isointense relative to gray matter on T1-weighted imaging and hypointense on T2-weighted imaging. No diffusion restriction of water was noted on either axial diffusion-weighted images or apparent diffusion coefficient maps. Both computed tomography and magnetic resonance imaging features suggested an intraorbital hemangioma. However, postoperative pathology (together with immunohistochemistry) identified an intraorbital granular cell tumor.

          Conclusions

          When intraorbital T2 hypointensity and free diffusion of water are observed on magnetic resonance imaging, a granular cell tumor should be included in the differential diagnosis of an intraocular tumor.

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          Most cited references12

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          Diffusion-weighted imaging of orbital masses: multi-institutional data support a 2-ADC threshold model to categorize lesions as benign, malignant, or indeterminate.

          DWI has been increasingly used to characterize orbital masses and provides quantitative information in the form of the ADC, but studies of DWI of orbital masses have shown a range of reported sensitivities, specificities, and optimal threshold ADC values for distinguishing benign from malignant lesions. Our goal was to determine the optimal use of DWI for imaging orbital masses through aggregation of data from multiple centers. Source data from 3 previous studies of orbital mass DWI were aggregated, and additional published data points were gathered. Receiver operating characteristic analysis was performed to determine the sensitivity, specificity, and optimal ADC thresholds for distinguishing benign from malignant masses. There was no single ADC threshold that characterized orbital masses as benign or malignant with high sensitivity and specificity. An ADC of less than 0.93 × 10(-3) mm(2)/s was more than 90% specific for malignancy, and an ADC of less than 1.35 × 10(-3) mm(2)/s was more than 90% sensitive for malignancy. With these 2 thresholds, 33% of this cohort could be characterized as "likely malignant," 29% as "likely benign," and 38% as "indeterminate." No single ADC threshold is highly sensitive and specific for characterizing orbital masses as benign or malignant. If we used 2 thresholds to divide these lesions into 3 categories, however, a majority of orbital masses can be characterized with >90% confidence.
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            Differentiation between benign and malignant orbital tumors at 3-T diffusion MR-imaging.

            To differentiate between malignant and benign orbital tumors at 3-T diffusion MR imaging. A retrospective study was conducted on 47 patients (34 males and 13 females aged 4-74 years) with orbital masses. They underwent echo-planar diffusion-weighted MR imaging of the orbit with b-factor of 0, 500, and 1,000 s/mm(2) at 3-T MR unit. Apparent diffusion coefficient (ADC) maps were reconstructed, and the ADC value of the orbital mass was calculated. The mean ADC value of the malignant orbital tumors (0.84 ± 0.34 × 10(-3) mm(2)/s) was significantly lower (P = 0.001) than that of the benign orbital tumors (1.57 ± 0.33 × 10(-3) mm(2)/s). The selection of an ADC value of 1.15 × 10(-3) mm(2)/s as a threshold value for differentiating malignant orbital tumors from benign lesions has a sensitivity of 95%, a specificity of 91%, and an accuracy of 93%. There was a significant difference in the ADC value between well- and poorly differentiated malignancies (P = 0.005). Apparent diffusion coefficient value at 3 T is an additional noninvasive imaging parameter that can be used for the differentiation of malignant orbital tumors from benign lesions, the characterization of some orbital tumors, as well as the grading of orbital malignancy.
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              Orbital masses: CT and MRI of common vascular lesions, benign tumors, and malignancies.

              A wide variety of space occupying lesions may be encountered in the orbit. CT and MR imaging frequently help confirm the presence of a mass and define its extent. Characteristic imaging features may help distinguish among lesions that have overlapping clinical presentations. This review focuses on some of the common orbital masses. Common vascular lesions that are reviewed include: capillary (infantile) hemangioma, cavernous hemangioma (solitary encapsulated venous-lymphatic malformation), and lymphangioma (venous-lymphatic malformation). Benign tumors that are reviewed include: optic nerve sheath meningioma, schwannoma, and neurofibroma. Malignancies that are reviewed include: lymphoma, metastasis, rhabdomyosarcoma, and optic glioma. Key imaging features that guide radiological diagnosis are discussed and illustrated.
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                Author and article information

                Contributors
                +886-2-28587158 , williamyuan.tw@gmail.com
                taichilin@hotmail.com
                jflirng@vghtpe.gov.tw
                wyguo@vghtpe.gov.tw
                fpchang@vghtpe.gov.tw
                mtho@vghtpe.gov.tw
                Journal
                J Med Case Rep
                J Med Case Rep
                Journal of Medical Case Reports
                BioMed Central (London )
                1752-1947
                13 May 2016
                13 May 2016
                2016
                : 10
                : 119
                Affiliations
                [ ]Division of Radiology, Taipei Municipal Gan-Dau Hospital (Managed by Taipei Veterans General Hospital), No.12, 225 Lane, Zhi-Sing Road, Taipei, Taiwan 11260
                [ ]Department of Radiology, Taipei Veterans General Hospital, No.201, Sec. 2, Shipai Rd., Beitou District, Taipei City, Taiwan 11217
                [ ]School of Medicine, National Yang Ming University, No.155, Sec.2, Linong Street, Taipei, 112 Taiwan
                [ ]Department of Ophthalmology, Taipei Veterans General Hospital, No.201, Sec. 2, Shipai Rd., Beitou District, Taipei City, Taiwan 11217
                [ ]Department of Pathology, Taipei Veterans General Hospital, No.201, Sec. 2, Shipai Rd., Beitou District, Taipei City, Taiwan 11217
                Article
                896
                10.1186/s13256-016-0896-5
                4866422
                27176551
                b770ac44-aa8a-4cff-9170-c80b4c3e37a0
                © Yuan et al. 2016

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 4 June 2015
                : 10 April 2016
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2016

                Medicine
                computed tomography (ct),magnetic resonance imaging (mri),orbit,granular cell tumor
                Medicine
                computed tomography (ct), magnetic resonance imaging (mri), orbit, granular cell tumor

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