An Unusual Tumor in an Uncommon Site-Orbital Rosai-Dorfman Disease: A Case Report

Rosai-Dorfman disease (RDD) is also known as Sinus Histiocytosis with Massive Lymphadenopathy. It is a rare, benign, self-limiting disease of phagocytic histiocytes presenting with massive painless cervical lymphadenopathy. RDD can present as a nodal disease and also extra-nodal involvement with episodes of exacerbation and remissions and relapses after treatment. Its etiology remains poorly understood and is highly variable in its clinical presentation and response to treatment. Its treatment is poorly defined but the prognosis is usually fair. Here we are reporting a rare, unusual clinical presentation of infraorbital soft tissue mass diagnosed as RDD with cyto-histopathological correlation. Only a few such cases have been reported in the literature.