Intracranial Rosai–Dorfman disease: Systematic review of published case reports and small series (2000–2025).

Abstract

e14108 Background: Intracranial Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that often mimics meningioma and lacks standardized management. We synthesized recent evidence on presentation, imaging/pathology, treatment, outcomes, and predictors of recurrence. Methods: This systematic review was conducted in accordance with PRISMA guidelines and a registered protocol (PROSPERO: CRD420251107147). PubMed, EMBASE, Scopus, and Web of Science were searched for case reports and case series (≤10 patients) describing histopathologically confirmed intracranial RDD. Two reviewers independently screened studies and extracted data. Descriptive statistics summarized patient, imaging, pathologic, treatment, and outcome variables. Group comparisons were performed using chi-square testing. Cox proportional hazards models were used to identify independent predictors of recurrence. Results: From 1,897 records, 176 studies including 261 patients were identified (226 adults and 35 children; 65.5% male). Magnetic resonance imaging was available in nearly all cases; 88.8% of lesions were dural-based, and 32.2% of patients had multiple intracranial lesions. Imaging typically demonstrated T1 isointensity, T2 iso- or hypointensity, and homogeneous enhancement. Lesions were described as meningioma-like in 82.0% of cases, and lytic bone erosion was reported in 41.8%. Histopathologic examination showed emperipolesis in 97.0% of cases, with S-100 and CD68 positivity and CD1a negativity in approximately 96%. Surgical resection was the primary treatment approach, with gross total resection (GTR) achieved in 58.0% of patients. Adjuvant corticosteroids, radiotherapy, and chemotherapy were administered in 33.6%, 16.7%, and 10.5%, respectively. Over a mean follow-up of 30.7 months, 84.2% of patients experienced functional improvement, recurrence occurred in 16.5%, and mortality was 5.7%. On multivariable analysis, the presence of multiple intracranial lesions was independently associated with higher recurrence risk (adjusted hazard ratio [HR] 5.08; 95% CI, 2.43–10.61; P < 0.001), while GTR was associated with a lower risk of recurrence compared with non-GTR surgery (adjusted HR 0.44; 95% CI, 0.21–0.89; P = 0.022). Conclusions: Intracranial RDD most often presents as a dural-based, meningioma-like mass and requires histopathologic confirmation for diagnosis. Surgical resection remains the mainstay of treatment; however, lesion multiplicity is the strongest predictor of recurrence, supporting the need for long-term surveillance. The role of adjuvant therapies remains uncertain, emphasizing the importance of multicenter registries, standardized reporting, and molecular profiling to guide future management.