Rosai-Dorfman disease presenting as a pulmonary artery mass

Ann Thorac Surg. 2010 Jan;89(1):300-2. doi: 10.1016/j.athoracsur.2009.05.016.

Abstract

Rosai-Dorfman disease is rare and typically presents with cervical lymphadenopathy, but may manifest as extranodal disease. This disease is generally indolent and self-limited, but it carries a poor or fatal prognosis when it is advanced or when it involves and compresses vital structures. We present a case of Rosai-Dorfman disease affecting the pulmonary arteries in a 22-year-old woman with severe, symptomatic right heart failure.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • Diagnosis, Differential
  • Female
  • Follow-Up Studies
  • Histiocytosis, Sinus / diagnosis*
  • Histiocytosis, Sinus / surgery
  • Humans
  • Pulmonary Artery*
  • Tomography, Emission-Computed, Single-Photon
  • Tomography, X-Ray Computed
  • Vascular Diseases / diagnosis*
  • Vascular Diseases / surgery
  • Vascular Surgical Procedures / methods*
  • Young Adult