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Editor,—The detection of a mucosa associated lymphoid tissue lymphoma in the orbit should motivate the ophthalmologist to a comprehensive systemic evaluation, given a significant association of this tumour with extranodal disease. We present the case of an orbital mass in a patient with leukaemia. Following excision and histopathological studies of the tumour, a diagnosis of MALT lymphoma was made, which led to the prompt evaluation and detection of extensive multiorgan involvement, and life saving therapy.
A 60-year-old man with a history of chronic lymphocytic leukaemia presented with a slowly growing mass in the right inferior orbit over the past year. Examination revealed a smooth, non-tender mass near the inferior orbital rim, distinct from the globe. Computed tomography showed a homogeneous tumour with smooth contours and no bony erosion (Fig 1). An excisional biopsy by anterior orbitotomy was subsequently performed. Histopathology revealed marginal zone cells and diffuse infiltration by plasma cells and plasmacytoid lymphocytes in a characteristic nodular cellular pattern, consistent with MALT lymphoma (Fig 2). Immunohistopathology showed the cells to be 60% B cell, and 40% T cell in origin. The patient was therefore promptly referred for colonoscopy, which disclosed multiple tumours, all consistent with MALT lymphoma. Two months later, the patient developed several cutaneous lesions on his arms, which again were MALT-type lymphoma by biopsy. He subsequently began chemotherapy and orbital radiotherapy, and has been doing well.
The mucosa associated lymphoid tissue lymphomas comprise part of a group of low grade B cell lymphomas presenting in the gut and other glandular epithelial tissues. These lymphomas differ from usual non-Hodgkin’s lymphomas in that they contain a variety of neoplastic B cells, rather than a monomorphous cell population. Histopathologically, these lymphomas contain small lymphocytes, plasma cells, and centrocyte-like cells, often in a reactive follicular pattern as shown here.1 MALT lymphomas may occur in a variety of extranodal sites, including breast, skin, kidney, and prostate.2 The common denominator of all these extranodal sites appears to be the glandular epithelium, for which these lymphomatous cells have a particular affinity.
Ocular adnexal MALT lymphomas are usually primary orbital tumours that typically carry a relatively small risk of mortality. They may, however, be associated with subsequent extraorbital disease involving other mucosal sites, as demonstrated here. The estimated time interval from presentation of an orbital mass to the development of an extraorbital lymphoma varies from 6 months to 5 years.34Furthermore, an estimated 25% of patients presenting with primary orbital lymphoma will develop extranodal disease during a 1 year follow up.3 This relatively long interval requires careful long term evaluation including serology, bone marrow biopsy, as well as chest and abdominal computed tomographies.
The often salient presentations of orbital masses necessitates serious evaluation. The detection of an orbital mucosa associated lymphoid tissue lymphoma, in particular, may be associated with systemic mucosal disease, and should direct the physician to the appropriate systemic studies
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