Extranodal Rosai–Dorfman Disease Arising in the Right Atrium

Abstract

Rosai-Dorfman disease is a rare, benign histiocytic proliferative disorder that commonly affects the lymph nodes. Although extranodal involvement has been reported in diverse sites, manifestation in the cardiovascular system is extremely uncommon. Specifically, the involvement of the heart by Rosai-Dorfman disease is an extraordinarily infrequent event.Here, the authors present a case of Rosai-Dorfman disease arising in the right atrium in a symptomatic 61-year-old man who initially presented with pleuritic chest pain and was found to have a large, lobulated, and circumscribed right atrial mass. The lesion exhibited an exuberant histiocytic and chronic fibroinflammatory process with focal emperipolesis within histiocytes. Immunohistochemical studies demonstrated strong S100 positivity in CD68+ CD1a− histiocytes. Although rare, Rosai-Dorfman disease should be considered in the differential diagnosis of a right atrial mass.

Primary pulmonary artery Rosai-Dorfman disease mimicking sarcoma

“…Surgical debulking was mandatory because of the lifethreatening localization. [3][4][5] Unilateral pneumonectomy seems more suitable to obtain oncologic radicality in the case of unilateral PA sarcoma, whereas a conservative procedure using pulmonary endarterectomy is indicated in the case of RDD. Therefore, an intraoperative histologic examination is crucial to plan the most appropriate surgical treatment.…”

“…Cardiovascular localization is life-threatening and associated with chest pain, severe dyspnea, and heart failure. [3][4][5] We describe the surgical treatment of a case of primary extranodal RDD involving the pulmonary artery (PA) and the apex of the heart.…”

Extranodal Rosai-Dorfman disease involving the right atrium in a 60-year-old male

“…RDD is a rare, idiopathic, non-neoplastic histiocytic disorder, and heart involvement is extremely rare. Only 9 cases involving the heart have been reported to date, 4 of which involved the atrium [2-4]. We here report one case of RDD involving the right atrium and provide a brief literature review, aiming to (i) avoid the trap of misdiagnosis of malignant or infectious diseases, (ii) emphasize the diagnostic histopathologic features of this disease, and (iii) achieve a better understanding of the character and treatment of this disease.A 60-year-old man presented with shortness of breath and fatigue.…”

Cardiothoracic manifestations of primary histiocytoses

“…Cardiac involvement in RDD is most often in the form of a myocardial mass. 32,35,39 On CT, these have often been described as poorly defined, but may have well-defined borders (Figure 7). On MR, these lesions appear hyperintense on T 2 weighted sequences and demonstrate delayed contrast enhancement (Figure 7).…”

“…14,32 However, up to 40% of patients present with extranodal involvement and in approximately 23% of patients, only extranodal disease is present. 35,36 Extranodal involvement most commonly includes the skin, bone and soft tissue. 33 Intrathoracic involvement is rare.…”