Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland

Purpose: Mucosa-associated lymphoid tissue lymphoma recently has been defin ed as a distinct subtype of non-Hodgkin's lymphoma with characteristic clin icopathologic features. A 37-year-old woman with systemic lupus erythematos us and unilateral mucosa-associated lymphoid tissue lymphoma of the lacrima l gland is described. Methods: The tumor was totally excised by lateral orbitotomy. Immunohistoch emical studies were performed with UCHL-1, CD20 (L26), leukocyte common ant igen, cytokeratin (CAM5), and kappa and lambda light chain antibodies. Results: The tumor was composed of centrocyte-like lymphocytes, cells with plasmacytoid features, and lymphoepithelial lesions. Most of the cells expr essed the CD20 protein and were positive for immunoglobulin kappa light cha in. The patient received no supplemental therapy. No systemic dissemination or local recurrence occurred during a follow-up of 26 months. Conclusion: The features of this case support the association between syste mic diseases and the subsequent development of mucosa-associated lymphoid t issue lymphoma.