Extranodal Rosai-Dorfman disease with ocular involvement in 5 cases with a literature review

AIM: To analyze the general profile, clinical symptoms, pathological features, and prognostic characteristics of extranodal Rosai-Dorfman disease (RDD) with ocular involvement. METHODS: This was a retrospective series of case study. Clinical data from 35 cases who had extranodal RDD with ocular involvement were collected for analysis, including 5 cases diagnosed at our hospital and 30 reported in the literature which searched via PubMed, the Chinese National Knowledge Infrastructure (CNKI), the Chinese Science and Technology Periodical Database (VIP), and WanFang Data database from database creation to April 2023. Lesion location, clinical presentation, pathological presentation, treatment modality, follow-up time, and prognosis were recorded. RESULTS: Lesions of five cases were located in the orbit, eyelid, lacrimal gland, or conjunctiva. The main presenting features were proptosis, eyelid swelling, and conjunctival hyperemia with decreased vision. Four patients underwent surgical resection, one received surgery and adjunctive immunosuppression, and none experienced recurrence during follow-up. A total of 30 cases were retrieved from the literature. The mean age was 41.4y, and 66.7% were male. The lacrimal gland and conjunctiva/subconjunctiva were the most commonly affected sites (each 20.0%). Most patients received surgical management (50.0%) or immunosuppressive therapy (20.0%). Only one recurrence (3.3%) was reported during follow-up. CONCLUSION: When symptoms like ocular protrusion and visual acuity loss occur, RDD should be considered in the differential diagnosis. The diagnosis of RDD primarily depends on pathological histology, which serves as the key basis for confirmation. Although RDD generally has a favorable prognosis, long-term follow-up of patients is still essential to closely monitor for potential recurrence.