Intracranial Rosai-Dorfman disease

Area of Science:

• Neurology
• Histopathology
• Oncology

Background:

• Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder.
• Central nervous system involvement is infrequent.
• Intracranial RDD often mimics other dural-based lesions like meningioma.

Observation:

• Five patients with intracranial RDD were diagnosed and treated.
• Preoperative misdiagnosis was common, with patients initially suspected of having meningioma or eosinophilic granuloma.
• Histopathology revealed characteristic emperipolesis with S-100 and CD68 positive histiocytes, and negative CD1a expression.

Findings:

• Surgical resection (total or subtotal) was performed on all five patients.
• No recurrence was observed in any patient post-surgery.
• Preoperative diagnosis remains challenging due to the lesion's dural-based nature.

Implications:

• Surgical resection is a highly effective treatment for intracranial RDD.
• Radiotherapy, steroids, and chemotherapy have shown limited therapeutic efficacy.
• Early and accurate diagnosis is crucial for effective management of intracranial RDD.