Intracranial ROSAI-DORFMAN Disease

Abstract

Rosai-Dorfman disease is a benign lymphohistiocytosis that often involve lymph nodes and present as massive lymphadenopathy with sinus histiocytosis. The disease is rarely associated with intracranial involvement. Herein, we report a 33-years-old man with recent onset of unconsciousness. According to his past medical history, he was suffering from frontal headache, ataxia and dizziness with no sensory or motor defect since August 2010. At initial work up, MRI showed infiltrating mass in the left parietal region. Microscopically, the mass consisted of infiltration of abundant lymphoplasma cells, neutrophils and some histiocytes scattered in fibrotic background. Emperipolesis (lymphocytophagocytosis) of histiocytic cells made the diagnosis of Rosai-Dorfman disease. Rosai-Dorfman disease should be added in the list of differential diagnosis for a dural mass mimicking meningioma or cerebral mass mimicking glioma, therefore, immunohistochemical staining for EMA, S100 and CD1a should be performed to rule out the differential diagnosis.

Keywords: Emperipolesis; intracranial Rosai-Dorfman disease.

Figure 1

Left periventricular mass that occupy temporal white matter and extend to adjacent gray matter

Figure 2

There is enhancing mass that shows subfalcine hernia to right side

Figure 3a

Lesion is composed of attenuated infiltrate of lymphoplasma cells, neutrophils and histiocytic cells with eosinophilic granular cytoplasm. Histiocytic cells occasionally show emperipolesis

Figure 3b

infiltrated plasma cells are immunoreactive for EMA

Figure 3c

Histiocytic cells with emperipolesis are immunoreactive for S-100 protein and negative for EMA