[Isolated intracranial Rosai-Dorfman disease: a case report and literature review]

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is an idiopathic histiocytosis that usually affects the lymph nodes. Occasionally it may affect the CNS, being exceptional intracranial involvement without lymph node lesions. In the absence of typical radiological signs, affected patients are generally operated under the suspicion of a meningioma. The histological diagnosis is obtained after the surgical procedure. It is a clinicopathological entity not well known, controversy exists about its pathogenesis, clinical course and therapeutic management.

We report the case of a 40-year-old male presented two generalized tonic-clonic seizures and brain MRI showed a left parieto-occipital extra-axial lesion extending into the posterior fossa, without presenting lesions at other levels. A partial resection of the lesion was performed and the histological findings were reported as Rosai-Dorfman disease.

Despite its low frequency, the ERD should be included in the differential diagnosis of dural-based masses, compared to more common, such as meningiomas. Due to lack of specificity of additional studies its diagnosis is fundamentally histologic. More research is needed to define the best therapeutic option.