Isolated cardiac involvement of Rosai-Dorfman disease

Rosai-Dorfman disease is a rare and multisystem disorder of unknown etiology. It commonly presents as cervical lymph node enlargement, but extranodal involvement may be presented in one-third of the cases. Usually, the clinical course of Rosai-Dorfman disease is benign but it can be malignant, both clinically and pathologically. Herein, we present an isolated cardiac case of extranodal Rosai-Dorfman disease without lymphadenopathy that involves the left ventricle in a symptomatic adult patient and a description of cardiac magnetic resonance imaging findings of this disease.