Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
October 2014
Next Case of the Month coming November 4 …
Rosai-Dorfman Disease
- Rare non-neoplastic disease of unknown etiology. Higher incidence in countries of the Far East.
- Usually presents in young children. Male predominance.
- Painless enlargement of cervical lymph nodes. Extranodal involvement in 40% of cases.
- Key Diagnostic Features:
- Massive cervical lymphadenopathy
- Homogeneously-enhancing lesions: dural-based, paranasal sinuses, adjacent soft tissues, and orbit (lacrimal gland and extraocular muscles)
- DDx:
- Other histiocytosis (eg, Erdheim-Chester disease)
- Lymphoma
- Leukemia
- Metastasis
- Rx:
- Systemic steroids
- Chemotherapy
- Possibly, symptomatic radiation and/or surgery
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