Isolated Cardiac Involvement in Rosai-Dorfman-Destombes Disease Causing Obstructive Shock

Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder that classically presents with painless massive lymphadenopathy; however, extranodal disease can occur.

A 63-year-old patient was incidentally found to have a soft tissue mass in the left atrium on computed tomography imaging during work-up for atrial flutter. After open surgery to obtain tissue diagnosis, the patient developed shock requiring venovenous extracorporeal membrane oxygenation support. The tissue biopsy revealed the diagnosis of RDD, and the patient received rituximab, which led to substantial clinical improvement.

We report a case of isolated cardiac RDD resulting in severe cardiac obstruction. Cardiac RDD is very rare, and a histopathologic diagnosis is required. Expert clinicopathologic correlation is required for early recognition and diagnosis.

RDD is a rare neoplastic disease with heterogeneous presentations, including cardiac involvement. Treatment can range from surgery to systemic therapy, including immunomodulatory agents and targeted therapy for patients with a mitogen-activated protein kinase mutation.