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Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 856-858
A clinicopathological analysis of orbital non-Hodgkins lymphoma from northern India

1 Department of Ocular Pathology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi, India
2 Department of Oculoplastics and Ocular Oncology Service, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi, India
3 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
4 Department of Radiology, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication27-Oct-2010

How to cite this article:
Sen S, Betharia SM, Dawar R, Sharma S, Kashyap S. A clinicopathological analysis of orbital non-Hodgkins lymphoma from northern India. Indian J Pathol Microbiol 2010;53:856-8

How to cite this URL:
Sen S, Betharia SM, Dawar R, Sharma S, Kashyap S. A clinicopathological analysis of orbital non-Hodgkins lymphoma from northern India. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Aug 5];53:856-8. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/856/72003


Extra-nodal non-Hodgkin's lymphoma accounts for 10-25% of the non-Hodgkin's lymphomas (NHL). [1] Eye is one of the most common sites of origin of extra-nodal non-Hodgkin's lymphoma (ENHL), and ocular adnexa accounts for 5-15% of the ENHL. Other sites include gastrointestinal tract, skin and respiratory tract. [2] A retrospective clinic-pathological analysis of ocular adnexal NHL over a 12-year period (1989-2001), from a tertiary eye care referral center in Northern India was undertaken as not much published data on orbital NHL is available from this part of the world.

A total number of 48 cases of orbital lympho-proliferative disorders were reviewed by light microscopy, of which, NHL accounted for 70.8% (34/48). There was marked male preponderance, with 28 males [Figure 1] and 6 females (4.5: 1), with a median age of 51.5 years (range 11 months to 75 years). Orbit was the commonest anatomical site, being involved in 91% (30/33) cases and the eyelid in 9% (3/33). Bilaterality was observed in 12% (4/33) and lachrymal gland involvement in 18% of the patients (6/33) [Table 1]. Systemic spread was observed in 12% patients (4/33) at presentation.
Figure 1: Clinical appearance of a case of NHL with axial proptosis, right eye

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Table 1: Clinicopathological profile of orbital NHL

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Computerized axial tomography showed the lesions to be generally homogenous and hyper-dense, intermediate to low signal intensity on T1W and proton density images; and hypo to hyper intense on T2W magnetic resonance (MR) images with homogenous enhancement [Figure 2]. Typically there was molding of the adjacent orbital structures without bony erosion or orbital expansion. Bone erosions were seen in 13.6% cases.
Figure 2: Coronal post contrast CT image to show a homogenously enhancing soft tissue mass involving the right lacrimal gland in a patient with lymphoma. The globe shows inferomedial displacement

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On light microscopy, (WHO classification), [3] 67.6 % of the cases (23/34) were extra-nodal marginal zone B-cell lymphomas of the MALT type (EMZL), followed by diffuse large cell lymphoma (DLBCL) 17.6% (6/34), Burkitt's lymphoma 5.9 % (2/34) and 2.9 % follicular centre lymphomas (FCL) (1/34). Plasmacytomas accounted for 5.9 % (2/34) cases. The EMZL consisted of predominantly small cells with varying degree of plasmacellular differentiation. Follicular colonization was a striking feature in which reactive follicles were seen to be infiltrated by tumor cells [Figure 3]. Lachrymal gland infiltration, giving rise to lympho-epithelial lesions was another hallmark of EMZL [Figure 4]. A follow up of 12 to 36 months was available in 18/34 cases (52.9%). Recurrence rate of 28 % (5/18) was noted, involving 3 cases of EMZL and a single case of DLBCL and FCL each [Table 1].
Figure 3: Extranodal marginal zone B-cell lymphoma, composed of small sized lymphoid cells. There is evidence of follicular colonization (H and E, ×200)

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Figure 4: Lymphoepithelial lesion in a case of maltoma, arrow points to a lacrimal duct (H and E, ×200)

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Immunohistochemistry (IHC) showed diffuse, strong positivity for B-cell (CD-20) in all the 34 cases [Figure 5]a. Monoclonality for k/ l light chains was observed in 50% (15/30) cases [Figure 5]b, [Table 1]. One of the 2 cases of plasmacytoma was positive for kappa light chain and the other was non-secretory type. The diagnosis of EMZL was confirmed by CD5, CD10 and Cyclin D-1 negativity in all the 23 cases.CD3 and CD68 positivity was observed focally in the non-neoplastic reactive cells and residual follicles.
Figure 5: a: CD-20 positive cells, confirming B-cell lineage (Avidin-biotin, ×400)
b: Monoclonality to show Κ light chain restriction (Avidin-biotin, ×200)

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Monoclonality has been reported to be between 25 to 88% in ocular NHL using various methods. [5] Mere absence of monoclonality by IHC thus does not rule out the diagnosis of NHL. Systemic spread in the present study was detected in 12% cases. The incidence of systemic lymphoma, both at presentation or follow-up, has been reported between 13-33%. Thus ocular extranodal lymphomas should be followed up for a minimum period of 5 years.

To conclude, NHL is the commonest orbital lympho-proliferative lesion in India as it is in other South-East Asian as well as in the Western countries. [4] Middle aged and elderly males with ages ranging between 48-63 years are usually affected. [1]

The imaging features of NHL are non specific as they often mimic metastatic tumours, primary lacrimal gland neoplasms and various other orbital pathologies including Grave's disease, optic nerve sheath tumours, orbital cellulitis. Light microscopy aided by immunohistochemistry is the gold standard for final diagnosis. The role of various aetiological infectious especially the role of Chlamydia psittaci in the pathogenesis of ocular NHL in India remains to be investigated. [6]

   Acknolwdgements Top

The authors wish to thank Ms Chanda Joshi for providing technical support for immunohistochemical staining. Financial support: Supported by grants from ICMR, New Delhi.

   References Top

1.Neri A, Jakobiec FA, Pellici PG, Dallafavera R, Knowles DM. Immunoglobulin and T cell receptor B chain gene rearrangement analysis of ocular adnexal lymphoid neoplasms: Clinical and biologic implications. Blood 1987;70:1519-29.  Back to cited text no. 1
2.Sasai K, Yamabe H, Dodo Y, Kashi S, Nagata Y, Hiraoka M. Non-Hodgkin's lymphoma of the ocular adnexa. Acta Oncol 2001;40:485-90.  Back to cited text no. 2
3.Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, et al. The World Health Organization classification of neoplasms of the hematopoietic and lymphoid tissues: Report of the Clinical Advisory Committee meeting--Airlie House, Virginia, November, 1997. Hematol J 2000;1:53-66.  Back to cited text no. 3
4.Coupland SE, Krause L, Delecluse HJ, Anangnotopoulas I, Foss HD, Hummel M, et al. Lymphoproliferative lesions of the ocular adnexa. Ophthalmology 1998;105:1430-41.  Back to cited text no. 4
5.Farmer JP, Lamba M, Lamba WR, Jordan DR, Gilberg S, Sengar DP, et al. Lymphoproliferative lesions of the lacrimal gland: Clinicopathological, immunohistochemical and molecular genetic analysis. Can J Ophthalmol 2005;40:151-60.  Back to cited text no. 5
6.Moslehi R, Devesa SS, Schairer C, Fraumeni JF Jr. Rapidly increasing incidence of ocular Non-Hodgkin lymphoma. J Natl Cancer Inst 2006;98:936-9.  Back to cited text no. 6

Correspondence Address:
Seema Sen
A-132, Defence Colony, New Delhi -110 024
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.72003

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1]

This article has been cited by
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Experimental and Molecular Pathology. 2016; 101(1): 74
[Pubmed] | [DOI]


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