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Diagnosis of multifocal extranodal Rosai Dorfman disease Case Study by fine needle aspiration cytology

Rosai-Dorfman Disease (RDD) or sinus histiocytosis with massive lymhadenopathy is a rare, nonneoplastic proliferative disorder of the cells of macrophage-histiocyte family having a self limiting course. Though it affects lymphnodes commonly, it can also involve many extranodal sites. These cases can often be misdiagn osed as lymphoma. Therefore, one has to be very careful not to interpret it as lymphoma or other causes of histiocytosis because of the difference in treatment protocol. Fine needle aspir tion cytology (FNAC) is a simple, fairly accurate diagnostic tool in the evaluation of such lesions. Although large numbers of RDD cases have been repor t d, review of the literature has revealed very few cases diagnosed by FNAC. Here, we report a case of RDD presenting with massive bilateral cervical and submandibular lympha denopathy along with unilateral orbital involvement, diagnosed by FNA cytology, which was s ubsequently confirmed by excisional biopsy and immunohistochemistry.