RESUMO
AIM: To study the pattern of presentation and management of nodal and extranodal primary RDD. METHODOLOGY: From 2010 to 2015, eleven patients diagnosed with Rosai-Dorfman Disease (RDD) presented at our institute. Nine of them were analyzed as they received treatment at our center. We had five neck primaries, three intracranial and one cutaneous disease. Hematological, radiological, histopathological and immunohistochemistry evaluation along with the treatment data was collected and analyzed. RESULTS: Nine cases treated at our institute had a median follow up of 41 months. Four of the five neck node primaries were treated with corticosteroids. Of the four, two had complete and two partial responses locally. Remaining one patient had partial response to surgery and corticosteroids. Three patients with CNS presentation treated with radiotherapy to a total dose of 20-40 Gy, had complete response locally. However, two patients had simultaneous neck nodes during presentation. Partial response was seen with corticosteroids alone in one and with radiation to the neck in the other patient, of which one succumbed to myocardial infarction within 3 months post treatment. Two of them received re-irradiation to the recurrent lesions with good results. CONCLUSION: Due to its rarity there is no particular guidelines regarding the management of patients with RDD. We conclude that low dose radiation either in combination with surgery or corticosteroids in nodal or extranodal lesions has shown a better local control.