To synthesize evidence on the epidemiology, presentation, imaging, histopathology, treatment strategies, and clinical outcomes of adult intracranial Rosai-Dorfman disease (RDD), addressing significant gaps in current understanding.
Key Findings:
Intracranial RDD often presents as a dural-based mass, commonly misdiagnosed as meningioma, highlighting the need for careful differential diagnosis.
Characteristic histopathological findings include large pale histiocytes with S100 and CD68 positivity and emperipolesis.
Surgical resection is the most common treatment, associated with symptomatic improvement but potential for recurrence.
Interpretation:
Adult intracranial RDD is underreported, with significant gaps in understanding its clinical and radiological features, necessitating further research to improve diagnosis and treatment.
Limitations:
Heterogeneity in patient selection and treatment approaches complicates outcome interpretation, and potential biases in included studies may affect reliability.
Many studies included were of low quality, affecting the reliability of pooled findings.
Lack of standardized management protocols for intracranial RDD.
Conclusion:
This systematic review highlights the need for improved understanding and reporting of adult intracranial RDD, emphasizing the critical importance of histopathological confirmation and tailored treatment strategies.
by Daniela A. Perez-Chadid, Aafreen Azmi, Jeremiah H. Wijaya, Temitope Oshinowo, Juan P. Avila-Madrigal, Aditi S. Gorthy, Sri Sai Lakshman Akkineni, Andrew Egladyous, Nemanja Novakovic, Morana Vojnic, Jonathan H. Sherman, Anil Nanda
