Clinical Scorecard: Intracranial Involvement in Adult Rosai-Dorfman Disease: A Comprehensive Review and Individual Patient Data Meta-Analysis of 327 Cases

At a Glance

Key Highlights

• Intracranial RDD presents predominantly as dural-based, avidly enhancing masses often mimicking meningiomas on imaging
• Diagnosis requires histopathology due to nonspecific radiologic features, characterized by emperipolesis and specific immunohistochemical markers
• Management is not standardized; surgical resection is primary therapy with adjuvant corticosteroids, radiotherapy, or systemic agents used in multifocal or residual disease

Guideline-Based Recommendations

Diagnosis

• Confirm diagnosis histopathologically with identification of large pale histiocytes showing emperipolesis
• Use immunohistochemical staining positive for S100 and CD68 and negative for CD1a to differentiate from other histiocytoses
• Consider intracranial RDD in differential diagnosis of dural-based enhancing masses, especially when meningioma is suspected

Management

• Pursue surgical resection for accessible or symptomatic lesions to obtain diagnosis and relieve mass effect
• Consider gross total resection when feasible to improve symptoms and reduce recurrence risk
• Use corticosteroids, radiotherapy, or systemic therapies for multifocal, residual, or surgically high-risk disease

Monitoring & Follow-up

• Monitor patients postoperatively for neurologic recovery and signs of recurrence or progression
• Employ follow-up imaging and clinical assessments due to risk of recurrence despite resection
• Tailor surveillance intensity based on extent of resection and presence of residual disease

Risks

• Recurrence or progression can occur even after gross total resection
• Misdiagnosis is possible due to radiologic similarity to meningioma and other entities
• Heterogeneity in treatment approaches complicates outcome prediction

Patient & Prescribing Data

Adult patients with histologically confirmed intracranial RDD

Surgical resection remains primary treatment; adjuvant corticosteroids, radiotherapy, and systemic agents are used variably with outcomes influenced by lesion accessibility and multifocality

Clinical Best Practices

• Obtain histopathologic confirmation with immunohistochemical profiling before definitive treatment
• Aim for gross total resection when safe to maximize symptomatic relief and reduce recurrence
• Use multidisciplinary approach to tailor adjuvant therapies in cases of multifocal or residual disease
• Maintain long-term follow-up with clinical and imaging evaluations to detect recurrence early
• Recognize the rarity and diagnostic challenges of intracranial RDD to avoid misdiagnosis and inappropriate management

References

• PRISMA Guidelines
• Joanna Briggs Institute Critical Appraisal Tools