Diagnosis of Multifocal Extranodal Rosai Dorfman Disease by Fine Needle Aspiration Cytology

Rosai-Dorfman Disease (RDD) or sinus histiocytosis with massive lymhadenopathy is a rare, non- neoplastic 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 misdiagnosed 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 aspiration cytology (FNAC) is a simple, fairly accurate diagnostic tool in the evaluation of such lesions.
Although large numbers of RDD cases have been reported, 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 lymphadenopathy along with unilateral orbital involvement, diagnosed by FNA cytology, which was subsequently confirmed by excisional biopsy and immunohistochemistry.