Extranodal Marginal Zone Lymphoma of the Ocular Adnexae in Children: Clinicopathologic Correlation

Abstract

Abstract: : Purpose: Extranodal Marginal Zone Lymphoma (EMZL) is the most common type of lymphoma that is present in the ocular adnexae. Extranodal marginal zone lymphomas have been described throughout the body and usually present in middle age (mean age 60). Recently, case reports of EMZL have been reported to occur in children in various systemic sites. Herein we describe the clinicopathologic characteristics of four patients who present with EMZL of the ocular adnexae. Methods: Four patients presented with lymphoid lesions present within the ocular adnexae. Each of the patients underwent biopsy of the lesions. Pathologic evaluation included: morphologic studies including light microscopy, immunohistochemistry, flow cytometry and gene rearrangement. Results: The patients were male and raged in age from 7 to 11 years of age. The lesions were present in the conjunctiva in three patients and in the caruncle in one patient. The lesions were present bilaterally in three patients and unilaterally in one patient. None of the patients presented with systemic involvement. Conclusions: EMZL occurs within the ocular adnexae in children and the clinicopathologic findings should be recognized.

Keywords: pathology: human • orbit • conjunctiva