Rosai-Dorfman disease (RDD) is a rare histiocytic disorder, usually presenting with massive lymphadenopathy. The involvement of extra nodal sites, bone and nodal sites like mediastinum are rare. Clinical data of three cases of extra nodal RDD who were admitted in our Pediatric Hemato-oncology unit were analyzed in the last 5 years. The extra nodal RDD (maxillary sinus, nasal pharyngeal focus, and external auditory meatus) were diagnosed based on histopathology and positron emission tomography (PET) scan. They were treated with steroids, but were refractory hence requiring salvage chemotherapy. Currently all 3 of them are in complete remission. Extra nodal RDD are difficult to diagnose, and histopathology and radiology play an important role. Here we present three cases, of which two were steroid refractory which were treated with salvage chemotherapy and are in complete remission.
Key words: Extra nodal, Rosai Dorfman disease, Children, Fluorodeoxyglucose Positron Emission Tomography.