Intracranial Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphomatous condition, predominantly involving lymph nodes. Rosai-Dorfman disease (RDD) (sinus histiocytes with massive lymphadenopathy) rarely affects the intracranial region without involvement of other sites. It is a rare and idiopathic histoproliferation disorder characterized by painless lymphadenopathy. We report a case of 43-year-old male who presented with unconsciousness; MRI was done and right temporofrontal mass was found. Excision was done, and on histopathology it confirmed RDD. 1. Introduction Rosai-Dorfman disease is a benign lymphohistiocytosis that often involves lymph nodes and presents as massive lymphadenopathy with sinus histocytiosis characterized by painless cervical lymphadenopathy, fever, leukocytosis, elevated ESR and polyclonal hypergammaglobulinemia. The disease was first described by Destombes in 1965 and later, in 1969, Rosai-Dorfman disease was first reported by Juan Rosai and Roland Dorfman as sinus histiocytosis with massive lymphadenopathy in young black males. RDD predominantly affected the children and young adult with mean age of 20.6 years but can be seen in the range of 1 to 74 years old. There is a slight male to female predominance (male？:？female = 1.4？:？1) [1]. The central nervous system can be involved in less than 5% [2]. Over 90% of patients present with cervical lymphadenopathy, and extranodal involvement including paranasal sinuses, skin, bone and Orbit which are seen in 43% of cases [3]. 2. Case Report 43-year-old male was referred to our hospital in December 2012, with history of new onset of sudden fainting attack at home for 8？min 4 days ago. He was unconscious for 8？min but with no seizure, no mouth frothing, no uprolling of eyes, no headache, no nausea, vomiting. At local hospital he has done MRI and found right temporofrontal lobe mass with edema. Four days later he came to our hospital in outpatient department of neurosurgery for further checkup. On the time of arrival in our hospital he was fully conscious and well oriented; GCS 15/15. He has never experienced this symptom before. There was no significant past history, no family history; hearts and lungs are clear. Vitals are within normal limit. Patient had normal movement, symmetrical pupil size, normal head wrinkle, normal facial expression and lymph node not palpable. On abdomen, spider nevi sign was seen. Muscle power was 5/5. Pathological sign was negative. He is smoker and consumes alcohol occasionally. MRI (Siemens 3.0 T) with contrast was repeated and showed right temoporofrontal lobe