Extranodal Rosai-Dorfman disease of the eye and ocular adnexa: A clinicopathologic series of 8 cases

Abstract

Purpose : To report the clinicopathologic findings of eight cases of extranodal Rosai-Dorfman disease of the eye and ocular adnexa.

Methods : In a non-comparative, consecutive case series, the database of Florida Lions Ocular Pathology Laboratory was searched for surgical specimens diagnosed with extranodal Rosai-Dorfman disease of the eye and ocular adnexa from the time-period of 1998 to 2018. The specimens were reviewed and the clinicopathologic features were evaluated.

Results : The diagnosis of extranodal Rosai-Dorfman disease of the eye and ocular adnexa was established by light microscopic examination of formalin fixed, paraffin embedded tissue from orbit, lacrimal gland, conjunctiva and cornea of seven patients. Six patients were male, and one was female. Mean age at the time of diagnosis was 19.1 years (range, 7-33 years). Six cases were unilateral, and two cases were bilateral (right = 5, left = 4, unknown = 1). One case was a recurrence. Four cases were from orbit, two were from lacrimal gland, one was from bulbar conjunctiva and one was from cornea. Findings on clinical presentation included masses of the eye and ocular adnexa, visual disturbances, lymphadenopathy, enlargement of the parotid gland, erythema and edema. The patients underwent debulking or excisional biopsy. Histopathologic examination revealed lymphocytes, plasma cells and histiocytes with foci of emperipolesis. Immunohistochemical stains including CD3, CD20, CD68, S100 and CD1A were performed.

Conclusions : Rosai-Dorfman disease is an uncommon, benign histiocytic disorder that rarely affects the eye and ocular adnexa. This is the largest single-center case series summarizing the clinicopathologic findings of extranodal Rosai-Dorfman disease of the eye and ocular adnexa. Herein we present the demographics, anatomic sites, clinical presentation, management, and histopathologic findings from eight cases of extranodal Rosai-Dorfman disease of the eye and ocular adnexa at the Bascom Palmer Eye Institute and Florida Lions Ocular Pathology Laboratory from 1998 to 2018.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.