Rosai-Dorfman Disease in a Rare Cardiac Presentation: Left Ventricular Mass Causing Ventricular Tachycardia

Rosai-Dorfman disease (RDD) is a rare, benign histiocytic disorder that most often presents with nodal involvement. Cardiac involvement is exceedingly uncommon and poses significant diagnostic and therapeutic challenges.

A 51-year-old woman presented with shortness of breath and palpitations and was found to have monomorphic ventricular tachycardia. Imaging revealed a left ventricular mass, and biopsy confirmed RDD. The lesion was surgically excised with cryoablation and CorMatrix reinforcement. The patient recovered uneventfully and remains disease free 25 months postoperatively.

RDD with cardiac involvement has been reported in only approximately 25 cases, with very few affecting the left ventricle. This case demonstrates how comprehensive imaging and deep myocardial biopsy are essential for accurate diagnosis. Surgical resection remains the treatment of choice for localized disease.

Cardiac RDD, though rare, should be included in the differential diagnosis of infiltrative cardiac masses. Complete excision offers curative potential.