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The Official Scientific Journal of Delhi Ophthalmological Society
Bilateral Lacrimal Gland Lymphoma
Namrata1, Rahul Ranjan2, Supriya Katiyar3, Anil Kumar Singh4
1Department of Ophthalmology, Sadguru Netra Chikitsalaya, Madhya Pradesh, India 
2Department of Radiodiagnosis, Rama Medical College, Uttar Pradesh, India 
3Department of Pathology, Rama Medical College, Uttar Pradesh, India 
4Department of Radiodiagnosis, Rama Medical College, Uttar Pradesh, India
Corresponding Author:
Rahul Ranjan 
MD, DNB
Associate Professor,
Department of Radio-Diagnosis,
Rama Medical College, Mandhana, 
Kanpur, Uttar Pradesh, India
Email id: rahulranjanradio10@gmail.com
Published Online: 30-JUL-2018
DOI: http://dx.doi.org/10.7869/djo.385
Introduction
Orbital lymphoma represents a small fraction of all systemic lymphomas that account for approximately 1–2 % of non- Hodgkin lymphomas. Lymphoma is reported to constitute between 6 and 8 % of orbital tumours, however, the incidence is estimated to be rising and has doubled in the last two decades.1 Lymphoproliferative disorders are the most common orbital masses in population more than 60 years of age. Lymphoproliferative disease includes a spectrum of disorders ranging from benign (lymphoid hyperplasia) to malignant disease (lymphoma), passing through atypical lymphoid hyperplasia. Immunohistochemical and molecular biological studies have been reliable to differentiate these entities. Orbital lymphomas are a heterogeneous group of malignancies, most of them are primary extranodal lymphoma of the marginal zone of mucosa associated with lymphoid tissue (MALT type lymphoma). These lymphomas arise in the lymphoid tissue acquired in certain extranodal sites as a result of chronic inflammation or autoimmune disorders. Recent advances in molecular and cytogenetics establish that lymphoma can be associated with Chlamydia psittaci infection.2 This tumour is seen more commonly in the 5th-7th decades of life with a slight female predominance. The lacrimal gland is in¬volved in nearly 40% of cases.3 The orbital infiltration by lymphoma is characterised by a palpable, firm or rubbery mass. Other symptoms are progressive proptosis, decreased visual acuity, motility disturbances and diplopia. Pain is an uncommon symptom of a lymphoproliferative lesion, in contrast to orbital pseudotumor, which manifests with acute pain.
The radiological findings found in orbital lymphoma have been previously reported in the literature. This tumour has been described as a mass with distinct margins, which shows an isointense signal on T1-weighted images and iso-hyperintense on T2-weighted images. Variable enhancement has been reported after contrast administration.4 Moreover, low values in the apparent diffusion coefficient on diffusion-weighted magnetic resonance imaging (MRI) have been found helpful to discriminate lymphoma from other expansile orbital lesions.5
On microscopy, lymphoproliferative lesions may be benign (reactive lymphoid hyperplasia) or malignant (lymphoma). Benign lesions demonstrate well-differentiated, somewhat pleomorphic lymphocytes, whereas malignant lymphoma demonstrates atypical cells with nuclear membrane abnormalities. The majority of orbital lymphomas are of B-cell origin.
In this communication, we present a 55 year old female who presented with a painless mass along the superolateral portion of bilateral orbits with proptosis and reduced mobility. MRI revealed mass lesions involving bilateral lacrimal glands extending posteriorly along the lateral margin of the orbit. Lesions were homogeneous appearing isointense on T1W (Figure 1), FLAIR images & iso to hypointense on T2W images (Figure 2) with restricted diffusion & reduced signal on ADC map (Figure 3). The lesion was hyperintense on STIR images (Figure 4). There was homogeneous enhancement on post contrast images. FNAC revealed dispersed population of medium to large sized atypical lymphoid cells suggestive of Lymphoproliferative disorder / Lymphoma of lacrimal gland origin (Figure 5).









References
  1. Bardenstein D. Ocular adnexal lymphoma: classification, clinical disease, and molecular biology. Ophthalmol Clin North 2005; 18:187–97.
  2. McKelvie PA. Ocular adnexal lymphomas: a review. Adv Anat Pathol 2010; 17:251-61.
  3. Demirci H, Shields CL, Karatza EC, Shields JA. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology 2008; 115:1626–1631. 
  4. Akansel G, Hendrix L, Erickson BA, Demirci A, Papke A, Arslan A, Ciftci E. MRI patterns in orbital malignant lymphoma and atypical lymphocytic infiltrates. Eur J Radiol 2005; 53:175–81.
  5. Politi LS, Forghani R, Godi C, Resti AG, Ponzoni M, Bianchi S, Iadanza A, Ambrosi A, Falini A, Ferreri AJ, Curtin HD, Scotti G. Ocular adnexal lymphoma: diffusion-weighted MR imaging for differential diagnosis and therapeutic monitoring. Radiology 2010; 256:565–74.
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Namrata, Rahul Ranjan, Supriya Katiyar, Anil Kumar SinghBilateral Lacrimal Gland Lymphoma.DJO 2018;29:72-73
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Namrata, Rahul Ranjan, Supriya Katiyar, Anil Kumar SinghBilateral Lacrimal Gland Lymphoma.DJO [serial online] 2018[cited 2018 Nov 17];29:72-73. Available from: http://www.djo.org.in/articles/29/1/Bilateral-Lacrimal-Gland-Lymphoma.html
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