The Teaching Point

Rosai-Dorfman disease is a rare entity and even fewer cases with cardiac involvement being reported. Imaging is important in the diagnosis and management of Rosai-Dorfman disease despite generally non-specific findings. Plain film, CT, and MRI imaging are extremely useful in detecting and characterizing masses, but are not specific for Rosai-Dorfman disease with a wide differential diagnoses including lymphoma, inflammatory and infectious processes. The radiologist should be aware of this rare entity and include it in the differential diagnosis.






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From the manuscript

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

Free full text article: 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.






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