Intracranial manifestations of adult Rosai-Dorfman disease: a systematic review and IPD meta-analysis of 327 cases

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Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytosis that involves the central nervous system (CNS) in approximately 5% of cases, most often presenting intracranially. While pediatric cases have been reported more frequently, adult intracranial RDD (IC-RDD) remains poorly characterized. We conducted a systematic review and individual patient data meta-analysis in accordance with PRISMA guidelines to define the epidemiology, clinical presentation, pathology, management strategies, and outcomes of adult IC-RDD. PubMed, Scopus, and the Cochrane Library were searched for histologically confirmed cases in adults over 18 years, and case reports, case series, and observational studies were included. Data were extracted on demographics, symptoms, imaging, histopathology, treatment, and outcomes. Primary endpoints were recovery and recurrence-free survival (RFS), with risk ratios (RRs) and 95% confidence intervals (CIs) calculated using random-effects models. A total of 327 patients from 186 studies met inclusion criteria. The median age was 43.6 years (range 18-83), with a male predominance (70.9%). The most common symptoms were headache (35.2%), seizures or loss of consciousness (28.7%), and visual disturbance (26.9%). Lesions were typically supratentorial intra-axial (52.9%) or extra-axial (20.5%). Surgical intervention was performed in 93.6% of patients, with gross total resection (GTR) achieved in 45.3%. At a median follow-up of 18.8 months, 37.9% achieved full recovery, 46.8% partial recovery, and 15.3% experienced recurrence, with a median RFS of 12 months. GTR was strongly associated with improved recovery (RR 0.26, 95% CI 0.19-0.37), whereas supratentorial intra-axial location (RR 0.56, 95% CI 0.41-0.75) and perilesional edema (RR 0.65, 95% CI 0.47-0.89) predicted poorer outcomes. These findings indicate that adult IC-RDD predominantly affects middle-aged men, presents with mass-effect symptoms, and has a location-dependent prognosis, with GTR conferring the best chance of recovery but recurrence remaining common and underscoring the need for long-term surveillance.

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