Rosai-Dorfman disease presenting as a pulmonary artery mass.

Walters, Dustin M; Dunnington, Gansevoort H; Dustin, Simone M; Frierson, Henry F; Peeler, Benjamin B; Kozower, Benjamin D; Ailawadi, Gorav; Jones, David R et al. · Ann Thorac Surg · 2010

case_report · Level V

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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.

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