Extranodal Rosai-Dorfman disease presenting as an isolated glenoid lesion in a high school athlete

Abstract

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), was first described as a clinical entity by Rosai and Dorfman in 1969. The first description of the disease reported a rare nonneoplastic disorder involving the proliferation of histiocytes predominantly involving the lymph nodes. ‘‘Sinus histiocytosis’’refers to histiocytosis that occurs in thedistended sinuses of lymph nodes, but since its initial description, RDD has been reported to involve a wide variety of organ systems and has been found in numerous locations throughout the body. RDD most commonly presents in young patients in the second or third decade of life, with an increased prevalence in men. RDD generally presents as bilateral, nontender, painless adenopathy in the neck, which may be accompanied by fever, elevated erythrocyte sedimentation rate, weight loss, and immunologic abnormalities such as leukocytosis and polyclonal hypergammaglobulinemia. Less frequently involved nodal sites are mediastinal, hilar, retroperitoneal, axillary, and inguinal. Extranodal RDDoccurs in 42% of patients, with 23% having isolated extranodal disease. Of the nearly 1000 patients with documented RDD, less than 3% have presented with isolated osseous involvement. In the 423 RDD cases presented by Foucar et al, 7.8% had osseous involvement, 2% had osseous involvement without lymphadenopathy, and 0.5% had isolated osseous