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Case No. 336
2014-05-01~2014-05-31
출제자 : 안성수
Hit : 322
| Period | 2014-05-01~2014-05-31 |
|---|---|
| Diagnosis | Rosai-Dorfman disease |
| Clinical information | F/59 CC : Headache for 1 year |
| Discussion | Imaging findings - Homogeneously enhancing masses on right parasagittal area, tuberculum sella and left cavernous sinus - Another enhancing mass between left lateral and medial pterygoid muscles - Intradural extramedullary masses showing T2 hypointensity and homogeneous enhancement < Rosai-Dorfman disease > • Sinus histiocytosis with massive lymphadenopathy • Rare benign histiocytic proliferative disorder Primarily involves lymph nodes Typically massive, painless cervical lymphadenopathy with fever, leukocytosis, ESR elevation 43% ; Simultaneous extranodal sites involvement Skin, eye, adnexa, paranasal sinuses, genitourinary system, CNS, bone, breast, soft tissues, thyroid Poorer prognosis than those with nodal disease • CNS involvement Rare (<5%) Approx. 50 cases of CNS Rosai-Dorfman disease have been reported in the literature up to August 2007 75% of cases involving the brain ; 25% involving the spinal canal Involvement of the CNS without concurrent nodular involvement is rare More than 90% of CNS Rosai-Dorfman disease cases involve the leptomeninges and seen as dural-based, contrast-enhancing masses |
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Correct AnswerSemi-Correct Answer |