Extranodal Rosai-Dorfman disease presenting as a cardiac mass in an adult: report of a unique case and lack of relationship to IgG4-related sclerosing lesions☆
Received 9 July 2009; accepted 20 July 2009. published online 07 December 2009.
Summary
Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare disease of unknown etiology that typically presents as nodal disease in young children. However, it also can present in various extranodal sites and can be difficult to recognize if not considered in the differential diagnosis. We describe a 55-year-old man who was discovered to have extranodal Rosai-Dorfman disease that presented as a cardiac mass involving the left atrium and ventricle during evaluation for atypical chest pain, and discuss the clinical, radiologic, and pathologic findings as well as treatment approach and consideration of a possible relationship of this entity to IgG4-related sclerosing lesions.
aLaboratory Department Naval Medical Center Portsmouth, Portsmouth, VA 23708, USA
bDepartment of Surgery, Naval Medical Center Portsmouth, Portsmouth, VA 23708, USA
cDivision of Hematopathology, Department of Pathology, Sentara Norfolk General Hospital, Norfolk, VA 23507, USA
dDivision of Anatomic Pathology, Mayo Medical Laboratories, Mayo Clinic- Rochester, Rochester, MN 55905, USA
Corresponding author.
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I am a military service member. This work was prepared as part of my official duties. Title 17 U.S.C. 105 provides that ‘Copyright protection under this title is not available for any work of the United States Government.’ Title 17 U.S.C. 101 defines a United States Government work as a work prepared by a military service member or employee of the United States Government as part of that person's official duties.
ABSTRACT