Primary Pulmonary Artery Rosai-Dorfman Disease Treated With Surgical Resection

Rosai-Dorfman disease (RDD) is a rare disease characterized by dilated lymph node sinuses with a large number of histiocytes. Extranodal disease is considered the uncommon subtype, which can be life-threatening when vital organs are involved. Here, we report a woman with a 6-month progressive dyspnea who visited our center. Computed tomographic pulmonary angiography revealed a filling defect in her bilateral pulmonary arteries. Bilateral pulmonary artery resection and reconstruction were performed, and postoperative pathologic analysis confirmed RDD. To our knowledge, this is the second case of extranodal RDD disease characterized by invasion of pulmonary artery treated with complete resection.