A CASE OF ROSAI-DORFMAN DISEASE PRESENTING AS A PULMONARY ARTERY MASS

ABSTRACT

SESSION TITLE Pathology Under the Microscope SESSION TYPE Case Reports PRESENTED ON 10/19/2022 1115 am - 1215 pm INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare, idiopathic, nonmalignant lymphohistiocytic proliferative disorder that presents with lymphadenopathy and less commonly with extranodal involvement (1). This is a case of a patient found to have a pulmonary artery mass and bone lesions consistent with RDD. CASE PRESENTATION A 33-year-old female with COVID pneumonia presented with one week of dyspnea, myalgias, and chills. She developed hypoxia requiring 2L of supplemental oxygen. Physical exam was benign and without lymphadenopathy. CT angiography demonstrated a well circumscribed 2.3cm x 2.1cm eccentric filling defect concerning for a pulmonary embolism versus vascular mass. She had a normal troponin and brain natriuretic peptide. Echocardiogram showed normal left ventricular ejection fraction and right ventricular size and function. Lower extremity dopplers were negative for acute deep venous thrombosis. Cardiac MRI demonstrated a mass in the posterior aspect of the proximal main pulmonary artery superior to the pulmonic valve measuring 1.9cm x 1.6cm that was consistent with a benign cardiac tumor. Patient was discharged and underwent sternotomy and excision of the mass one week later. Pathology showed histiocytosis consistent with RDD. Post-operatively she developed recurrent fevers and imaging showed bony lesions in her lumbar spine, maxilla, and skull base. Pathology from an IR guided biopsy of the lumbar lesion was suggestive of RDD. DISCUSSION: RDD is a rare, nonmalignant lymphohistiocytic proliferative disorder that usually involves lymph nodes. Concurrent nodal and extranodal involvement has been reported in 43% of cases while isolated extranodal involvement has been reported in 23% of cases. Common extranodal sites include cutaneous, soft tissue, upper respiratory tract, bone, and central nervous system (1). There are only a few cases reported of pulmonary artery involvement. These cases include a patient with RDD invading the pulmonary trunk and aorta who required surgical resection and reconstruction due to impending right ventricular failure (2) and a young woman with RDD causing nearly complete obstruction of the main pulmonary artery resulting in severe pulmonary hypertension and heart failure who required debulking (3). This case demonstrates RDD involving the main pulmonary artery and bones which was incidentally discovered when the patient was hospitalized for COVID pneumonia. RDD has a benign course but when the pulmonary artery is involved, patients often require surgical excision. CONCLUSIONS: RDD is a benign proliferation of histiocytes that most commonly presents with cervical lymphadenopathy. Extranodal involvement has been reported but pulmonary artery involvement is rare. RDD has a benign course, but pulmonary arterial involvement often requires surgical excision. Reference #1 Gaitonde, S. (2007). Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy an overview. Archives of pathology & laboratory medicine, 131(7), 1117-1121. Reference #2 Prendes, B. L., Brinkman, W. T., Sengupta, A. L., & Bavaria, J. E. (2009). Atypical presentation of extranodal Rosai-Dorfman disease. The Annals of thoracic surgery, 87(2), 616-618. Reference #3 Walters, D. M., Dunnington, G. H., Dustin, S. M., Frierson, H. F., Peeler, B. B., Kozower, B. D., … & Lau, C. L. (2010). Rosai-Dorfman disease presenting as a pulmonary artery mass. The Annals of thoracic surgery, 89(1), 300-302. DISCLOSURES No relevant relationships by Veena Dronamraju Advisory Committee Member relationship with Nabriva Please note 1 day Added 03/14/2022 by Rohit Gupta, value=Consulting fee No relevant relationships by MARUTI KUMARAN no disclosure on file for Bilal Lashari;No relevant relationships by Parth Rali No relevant relationships by Stephanie Tittaferrante No relevant relationships by Yoshiya Toyoda