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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 407-408
Mucosa-associated lymphoid tissue lymphoma in conjunctiva

Department of Pathology, Medical College, Kolkata, West Bengal, India

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A case of primary mucosa-associated lymphoid tissue (MALT) lymphoma (marginal zone B-cell lymphoma of MALT according to WHO classification) in conjunctiva, which presented as a slowly growing salmon-colored mass at limbus of left eye is reported. Histological examination revealed a diffuse low-grade lymphoma. Immunohistochemical analysis using monoclonal antibodies showed that the tumor cells are leukocyte common antigen (CD45)+, CD20+, CD3−, CD5−, CD10− and CD43−, which confirmed the B-cell lineage of lymphoma. The case is being reported for its rarity and clinical importance of recognizing such cases because of excellent prognosis.

Keywords: Conjunctival biopsy, mucosa-associated lymphoid tissue lymphoma

How to cite this article:
Mondal SK. Mucosa-associated lymphoid tissue lymphoma in conjunctiva. Indian J Pathol Microbiol 2008;51:407-8

How to cite this URL:
Mondal SK. Mucosa-associated lymphoid tissue lymphoma in conjunctiva. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 Jul 3];51:407-8. Available from: http://www.ijpmonline.org/text.asp?2008/51/3/407/42539

   Introduction Top

Lymphocytes are present in the substantia propria of the conjunctiva and are scattered among the acini of the lacrimal gland; these sites are grouped with mucosa-associated lymphoid tissue (MALT). MALT is seen most notably in Waldeyer's ring, Peyer's patches of the intestine and the appendix and is typified by reactive follicles, prominent marginal zones and subepithelial aggregates of plasma cells. Using these criteria as well as immunohistochemical similarities, lymphoma with MALT-like characteristics have been noted to occur in increased frequency at certain body sites including the stomach, breast, lung, thyroid gland, salivary gland and ocular adnexa. [1]

   Case History Top

A 46-year-old male patient attended ophthalmology outpatient department with the complaint of dryness and a small swelling in his left eye, which increased very slowly. There was no past history of fever, weight loss, or lymphadenopathy.

On examination, a salmon-colored mass was found at left limbus on the nasal side [Figure 1]. The tumor was readily movable over the epibulbar surface and did not cause extraocular motility problems or proptoses. No lymphadenopathy or intraabdominal organomegaly was found. Chest and cardiovascular examination was normal. Routine hematological, biochemical and relevant investigations were within normal limits. A provisional clinical diagnosis of primary lymphoid tumor of conjunctiva was made.

Pathological findings

Biopsy taken from conjunctival lesion showed a fish-flesh appearance of the lesion. Microscopical examination revealed diffuse sheets of lymphoid cells. The cells were predominantly composed of small lymphocytes [Figure 2]. Follicles or germinal centers were not found. A histopathological diagnosis of MALT lymphoma (diffuse low-grade B-cell lymphoma) was given. Immunohistochemistry revealed that the tumor cells expressed CD45 and CD20. They were negative for CD3, CD5, CD10, CD23 and CD43. Thus, the B-cell lineage of MALT lymphoma was established. [2]

Lymph node screening and whole body scan did not reveal any organ or lymph node involvement. Bone marrow examination revealed no involvement. Diagnosis of primary MALT lymphoma (diffuse low-grade B-cell lymphoma) was established in view of absence of any other lymph node, organ, or bone marrow involvement by the tumor cells.

The patient was given low-dose radiotherapy (photons/electrons) in a dose of 30 Gy. A 3-year follow-up showed a remarkable improvement with shrinkage of mass and the patient remained symptom-free. The disease remained localized and did not show systemic involvement.

   Discussion Top

Malignant lymphoma commonly originates from lymph nodes, spleen and bone marrow. Less frequently, it primarily involves gastrointestinal tract, lungs, skin, orbit, brain, thyroid and breast. Primary lymphoma in conjunctiva is rare. While extranodal lymphoma expresses the monotypic immunoglobulin and pan-B antigens, CD23 and CD43 may or may not be positive. It fails to express CD5, CD3 and CD10. Furthermore, extranodal lymphoma follows a more indolent course than nodal lymphoma.

Mucosa-associated lymphoid tissue developing in response to chronic infection or autoimmune disease has been recognized as a possible site of origin for a distinct type of B-cell lymphoma. Histologically, MALT-type lymphomas are heterogeneous, covering a cytological spectrum ranging from small lymphocytes to centrocyte-like cells or monocytoid B cells. Wenzel et al. suggested that MALT lymphoma might express CD5 antigen rarely and aberrant CD5 expression had been thought to be a marker for early dissemination, aggressive clinical course and possibility of relapse after local radiotherapy. [3] Likewise, CD43 positive ocular lymphomas are associated with a higher rate of subsequent distant recurrences and the rate of lymphoma-related death as found by Nora et al. [4]

The treatment modalities for ocular lymphoma are surgical excision, local radiotherapy and chemotherapy. Dose-response data suggest that radiation dose for MALT lymphoma of the ocular adnexa is 30.6-32.4 in 1.8 fractions and follicular lymphoma is adequately controlled with doses in the mid-20 Gy range. In the group of patients with extranodal marginal zone B-cell lymphoma of MALT (B-EMZL according to WHO classification), the estimated 5-year overall survival was 92.9±6.6%. [5] Shields et al. , by univariate analysis, observed factors predictive of systemic lymphoma were site (tumor at an extralimbal site, i.e., fornix or midbulbar conjunctiva) and increasing number of conjunctival tumors. [6] They found lymphoid tumors of the conjunctiva are associated with systemic lymphoma in 31% of patients.

   References Top

1.Harris NL. Extranodal lymphoid infiltrate and mucosa-associated lymphoid tissue (MALT): A unifying concept. Am J Surg Pathol 1991;15:879-84.  Back to cited text no. 1  [PUBMED]  
2.Zukerberg LR, Medeiros LJ, Ferry JA, Harris NL. Diffuse low-grade B-cell lymphomas: Four clinically distinct subtypes defined by a combination of morphologic and immunophenotypic features. Am J Clin Pathol 1993;100:373-85.  Back to cited text no. 2  [PUBMED]  
3.Wenzel C, Dieckmann K, Fiebiger W, Mannhalter C, Chott A, Raderer M. CD5 expression in a lymphoma of the mucosa-associated lymphoid tissue (MALT)-type as a marker for early dissemination and aggressive clinical behaviour. Leuk Lymph 2001;42:823-9.  Back to cited text no. 3    
4.Nora M, Lukenda A, Bonmann M, Kalauz M, Petrovecki M, Bollmann R. Outcome and prognostic factors in ocular adnexal lymphoma. Croat Med J 2004;45:328-32.  Back to cited text no. 4    
5.Fung CY, Tarbell NJ, Lucarem MJ, Goldberg SI, Linggood RM, Harris NL, et al . Ocular adnexal lymphoma: Clinical behavior of distinct World Health Organization classification subtypes. Int J Radiat Oncol Biol Phys 2003;57:1382-91.  Back to cited text no. 5    
6.Shields CL, Shields JA, Carvaino C, Rundle P, Smith AF. Conjunctival lymphoid tumours: Clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology 2001;108:979-84.  Back to cited text no. 6    

Correspondence Address:
Santosh Kumar Mondal
C/o Jogeswar Ghosh, 872 Rabindranath Tagore Road, Bediapara, Dumdum, Kolkata - 700 077, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.42539

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