Rare Presentation of Sinonasal Mass: Extra-Nodal Rosai Dorfman Disease: A Case Report

Introduction: Rosai -Dorfman disease (RDD) is a rare, usually self-limiting, benign condition which is characterised by over production and accumulation of a histiocytes in the lymph nodes resulting in painless cervical lymphadenopathy. Extra -nodal RDD is uncommon and have been reported in approximately 43 % of cases and can affects skin, respiratory tract, a solitary bone, nasal cavity, or the central nervous system. Histopathological examination plays an important role in diagnosis which shows emperipolesis. Denitive treatment yet to established due to its rarity and propensity for spontaneous remission. We reported a case of extra-nodal RDD involving the Case report: sinonasal area with intracranial extension without lymphadenopathy in a 55 years old male who presented with complaint of nasal obstruction with difculty nasal breathing and later was diagnosed of Rosai Dorfman disease on histopathological examination. Patient was managed by surgical excision and adjuvant treatment with oral steroid and methotrexate. Due to lack of pathognomonic clinical and rad Conclusion: iological diagnostic features it is difcult to diagnose RDD involving sinonasal area hence it is important to keep RDD as one of differential diagnosis in benign sinonasal mass. Histopathology examination plays a very important role in denitive diagnosis of RDD. Surgical intervention is only recommended when there is cosmetic deformities and obstructive symptoms. Long-term follow up is necessary to recognise and prevent the recurrences of disease.