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Pathology Forum
December 1999

Quiz Case 2

Author Affiliations


Arch Otolaryngol Head Neck Surg. 1999;125(12):1399-1401. doi:

Sinus histiocytosis with massive lymphadenopathy, (Rosai-Dorfman disease), is a rare cause of lymph node enlargement in children. In 1969, Rosai and Dorfman1 described 4 cases of sinus histiocytosis with distinct clinical and pathologic features that had previously been classified as reticuloendotheliosis.

Most patients present in the first decade of life, with 80% of cases occurring in patients younger than 20 years. There is a slight male predominance. The disease occurs equally in blacks and whites and less commonly in Asians.2

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