Extranodal Rosai-Dorfman disease involving the right atrium in a 60-year-old male
© Bi et al.; licensee BioMed Central Ltd. 2014
Received: 24 April 2014
Accepted: 2 June 2014
Published: 9 June 2014
Rosai-Dorfman disease (RDD) involving the cardiovascular system is extremely rare; to our knowledge, there are only 9 cases in the literature. Here, a case of a 60-year-old male with RDD involving the right atrium is presented. A comprehensive literature review was undertaken to summarize the clinical and pathologic features of this disorder.
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2143194139120169.
KeywordsRosai-Dorfman disease Right atrium Differential diagnosis
Letter to the editor
To date, approximately 7 months of routine follow-up after the surgery has been performed; the patient has not received any further treatment and remains well, with no recurrence.
The involvement of an extranodal site in association with lymphadenopathy is present in approximately 43% of all RDD cases, and only 23% of cases show isolated extranodal RDD. The skin, soft tissue, and upper respiratory tract are the most frequently affected sites. The 4 reported cases involving the right atrium included 2 males and 2 females ranging in age from 40 to 79 years. Two patients presented with chest pain, whereas the other two cases were incidentally diagnosed during autopsy. The sizes of the lesions ranged from 0.6 to 5 cm, showing variable numbers of pale-staining histiocytes with emperipolesis and a lymphoplasmacytic infiltrate on a background of fibrosis. Our case showed similar histologic findings associated with prominent fibrosis and rare emperipolesis. The histopathologic features of RDD at extranodal sites reflect the fact that fibrosis tends to be more pronounced and lymphocytophagocytosis less conspicuous than the nodal disease, which makes extranodal diseases more difficult to identify histopathologically. The immunohistochemical staining of lymph nodes and extranodal sites are similar to what has been previously reported. The immunohistochemical staining of our case showed S-100 and CD68 positivity in histiocytes, which is similar to the features of nodal disease. The lack of IgG4-positive plasma cells ruled out IgG4-related sclerosing disease. Of the two living patients with right atrial involvement who have been described in the literature, one took steroids, and both disclosed no evidence of disease progression. In most cases, RDD undergoes quick and complete spontaneous resolution. However, the involvement of vital organs can be associated with a potentially fatal outcome. The prognosis of atrial RDD remains unclear because cardiovascular system involvement is extremely uncommon.
The differential diagnoses of a cardiac mass with microscopic features similar to those of RDD include Langerhans cell histiocytosis (in which the cells are positive for both S-100 protein and CD1a), an inflammatory myofibroblastic tumor (which has a background proliferation of spindle cells associated with an infiltrate of mononuclear inflammatory cells and ALK-1 positivity), metastatic malignant melanoma (in which cells are positive for Melan-A), Hodgkin’s disease (which shows characteristic Reed-Sternberg cells and positivity for CD15 and CD30), and fungal or mycobacterial infections (which are validated by positive staining with GMS, PAS, and acid-fast stain).
RDD is a rare entity, and RDD with cardiac involvement is extremely rare. Doppler echocardiography, computed tomography, and magnetic resonance imaging are useful in detecting and characterizing masses but are not specific for RDD. Pathologists must be aware that RDD should be considered among the possible differential diagnoses for cardiovascular tumors.
Written informed consent was obtained from the patient prior to publication of this case report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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