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Radiology Quiz Case 2: Diagnosis
Arch Otolaryngol Head Neck Surg. 2009;135(3):319.
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Diagnosis: Extranodal Rosai-Dorfman disease involving the right orbit and nasal cavity
Rosai-Dorfman disease, a generally benign entity characterized by sinus histiocytosis with massive lymphadenopathy, was first described by Rosai and Dorfman1 in 1969. The disease, which occurs primarily in children and young adults, mostly presents with massive cervical lymphadenopathy (90%), fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia.2 When it affects nonlymphoid organs, it is classified as extranodal Rosai-Dorfman disease. Extranodal disease occurs in 43% of patients, and 75% of the extranodal lesions appear in the head and neck region.2 Orbital involvement, which is characterized by exophthalmos, ptosis, conjunctival injection, diplopia, and dry eyes,3 has been reported in 11% of the extranodal cases. Concomitant involvement of the orbit and nasal cavity has been documented in very few reports.4-5
The origin of extranodal Rosai-Dorfman disease remains unclear. Several possible mechanisms, including occult chronic infections, an aggressive autoimmune response to an infectious agent, and agents that can cause histiocytes to proliferate, have . . . [Full Text of this Article]
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Radiology Quiz Case 2
Chia-Hsiang Fu, Shiang-Fu Huang, Shih-Ming Jung, Shy-Chyi Chin, Chun-Ta Liao, and I-How Chen
Arch Otolaryngol Head Neck Surg. 2009;135(3):317.
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