Extranodal Rosai-Dorfman Disease Presenting as an Epibulbar Tumor

Abstract

Although extranodal manifestations are common in RDD and the lack of lymph node involvement is rare, our study supports that whenever there is an epibulbar tumor as a manifestation of RDD, the absence of lymphadenopathy is characteristic. Only 2 of the 9 reported cases presented with lymphadenopathy. The presence of emperipolesis and S-100-positive histiocytes during histopathologic evaluation confirms the diagnosis even in the absence of lymphadenopathy.

Bilateral Epibulbar Xanthomas—A Case Report

“…16 Isolated epibulbar masses are a rare but recognized manifestation of extranodal Rosai-Dorfman disease. [6][7][8][9] Bilateral, multiple, subconjunctival nodules as an isolated finding in Rosai-Dorfman disease has been reported in a child and bilateral epibulbar masses have been reported as a presenting feature in an adult with Rosai-Dorfman disease and systemic involvement. 8,9 Epibulbar involvement has been described in disseminated Langerhans cell disease and an isolated limbal lesion in Langerhans cell disease has been reported in adult.…”

“…Rarely, epibulbar involvement may occur as a presenting feature or as an isolated finding. [3][4][5][6][7][8][9][10] Epibulbar xanthomatous lesions are extremely rare, and to our knowledge, the occurrence of bilateral multiple lesions, in the absence of other features of a histiocytic disease, has not been reported previously.…”

Subconjunctival Masses Associated With Central Nervous System Rosai–Dorfman Disease

“…As in our patient, the majority of cases of ocular and CNS Rosai-Dorman did not have concomitant lymphadenopathy. 11,12 Interestingly, our patient had a history of cervical node swelling suggesting that he initially had intranodal disease that regressed and later manifested itself in the eye and the CNS.…”

Atypical Rosai-Dorfman Disease with Lacrimal Gland Involvement

“…11 Fernandes et al presented a patient with a limbal tumor associated with RDD that underwent a superficial sclerokeratectomy and had no recurrence during 14 months of follow-up. 12 If surgery is not an option in some cases, systemic steroids and radiation may be associated with a favorable outcome. 13 In the case reported here, surgical excision was used in a patient with a good prognosis.…”