Rosai-Dorfman disease (RDD) is a rare form of non-Langerhans histiocytosis. It is often idiopathic in etiology, but has been associated with viral, autoimmune, and malignant disease. Adequate diagnosis of RDD requires a combination of clinical symptoms, radiography, and histology. Most commonly, patients with RDD present with cervical lymphadenopathy. We describe a case of a young female who was initially thought to have a pulmonary embolism at the time of a COVID-19 infection but was noted to have a rare occurrence of RDD presenting as a pulmonary artery mass upon further evaluation of radiology and histology. Though RDD is frequently benign, extranodal involvement can progress to end organ damage and must be recognized appropriately.
Keywords: extranodal involvement; non‐Langerhans cell histiocytosis; pulmonary artery filling defect.
© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.