Sinus histiocytosis with massive lymphadenopathy: a "massive" misnomer.

Sinus histiocytosis with massive lymphadenopathy also known as Rosai-Dorfman disease is a rare, benign, histiocytic disorder of unknown origin characterized by lymphadenopathy. Since its original description by Rosai and Dorfman in 1969, small number of cases has been reported; hence no specific diagnostic criteria and treatment guidelines have been suggested. The purpose of this article is to present and discuss a case of Rosai Dorfman Syndrome in 4-year-old child diagnosed with the help of Fine Needle Aspiration Cytology. The swelling resolved over a period of 18 months without any recurrence in 2-year follow-up period. This case report highlights the role of Fine Needle Aspiration Cytology in the diagnosis of Rosai-Dorfman disease and the importance of regular follow ups using a careful wait and watch approach in its management. The report also briefly discusses the various dilemmas associated with its diagnosis and treatment.