A case of Rosai-Dorfman Disease in a pediatric patient with cardiac involvement

Abstract

Rosai-Dorfman disease (RDD) involves abnormal proliferations of oddly behaving histocytes that are not derived from the Langerhan's Cell linage. These collections tend to occur within lymph nodes, with occasional extra nodal presentation. While RDD is a rare entity itself, extra nodal cases are even more so, with even fewer reporting cardiac involvement, and previously only in adults. This report describes the disease in a pediatric patient who had the unique feature of an extra nodal cardiac mass. The patient, who was known to have sickle cell disease, was initially erroneously thought to have acute chest syndrome. Sudden changes in the patient's status, including development of 3rd degree heart block, demanded investigation with additional imaging. Chest CT revealed a mass arising from the cardiac interatrial septum and encircling the entire thoracic aorta. Imaging features of Rosai-Dorfman disease are nonspecific, complicating the diagnosis. We present this case with discussion of this extremely uncommon entity. We describe the diagnostic methods, the differential diagnosis, and the treatment options.

Rosai-Dorfman Disease as Constrictive Pericarditis: An Unusual Childhood Presentation

“…Two patients had sickle cell disease and a similar presenting feature in the form of an intracardiac mass arising from the interatrial septum, associated with infiltration in other parts of the heart. Both had conduction abnormalities and required permanent pacing of the heart [ 8 , 9 ]. The other case involved tricuspid and pulmonary valves along with multiple extranodal site involvement, which was diagnosed during an autopsy [ 10 ].…”

Interatrial Septal Mass Secondary to Rosai-Dorfman Disease

“… 2 This usually manifests as an intracardiac mass and, in the cases reported, often represents multifocal underlying cardiac involvement. 2 From an electrophysiological standpoint, there is report of 2 children with RDD manifesting with complete heart block 3 , 4 and 1 adult presenting with an electrical storm. 5 …”

18F-FDG PET/CT Imaging of Extranodal Rosai-Dorfman Disease with Hepatopancreatic Involvement - A Pictorial and Literature Review

“…Extranodal involvement has been noted in more than 40% of cases, and cutaneous lesions represent the most common form of the extranodal disease [ 2 ]. There have been a few reports of extranodal RDD involving the nervous system, heart, and other organ systems [ 3 - 4 ].…”