Rosai-Dorfman Disease: A 30-Year Institutional Study of Clinical Features, Treatment Patterns, and Outcomes

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ABSTRACT Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis with limited data guiding management. This study aimed to characterize the clinical, treatment, and outcome features of patients with RDD. We retrospectively reviewed 65 individuals with histopathologically confirmed RDD from 1994-2025 at a single institution. Demographic, clinical, treatment, and outcome data were analyzed. Overall response (OR) was defined as complete or partial response. Median age at diagnosis was 46 years; 82% had extranodal disease, 63% had localized involvement, and 29% presented with cutaneous disease. Initial treatments included surgery (n=32), observation (n=13), corticosteroids (n=10), systemic therapy (n=9), and radiotherapy (RT; n=1). Thirty-eight percent of observed patients experienced spontaneous complete or partial regression. Initial OR rates were 80% for corticosteroids and 22% for systemic therapy. Initial surgery resulted in complete resection in 72% of patients. The single individual treated with initial RT achieved a partial response. Twenty-six individuals (40%) progressed after initial treatment. Nine patients received RT (20-38 Gy) during their disease course. Among 11 evaluable lesions, best responses included one complete response, six partial responses, three stable disease, and one progression. Four had no post-RT progression. No RT late effects were observed. At median follow-up of 56.2 months, 17% required hospitalization, mostly due to mass effect. Six individuals (9%) died of unrelated causes. In the largest single-center retrospective cohort to date, RDD demonstrated heterogeneous behavior and treatment responses but generally indolent courses. RT, though used in a small patient subset, showed promising local activity with minimal toxicity.

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