NEUROLOGY 2008;70:e42
© 2008 American Academy of Neurology
Resident and Fellow Section
Teaching NeuroImage: Isolated intracranial Rosai-Dorfman disease mimicking a meningioma
Brett J. Theeler, MD,
Joren B. Keylock, MD and
Stephen M. Yoest, MD
From Madigan Army Medical Center, Department of Neurology, Tacoma, WA.
Address correspondence and reprint requests to Captain Brett J. Theeler, USAMC, Madigan Army Medical Center, Neurology Service, 9040A Fitzsimmons Dr., Tacoma, WA 98431 btheeler{at}hotmail.com
A 56-year-old woman presented with involuntary left arm and leg movements. MRI with gadolinium revealed a heterogeneously enhancing, extra-axial mass at the right frontoparietal convexity, causing mass effect, vasogenic edema, and dural thickening (figure). Intraoperatively the mass was noted to invade nearby cortex, sulci, and the superior sagittal sinus. Meningioma was suspected. Pathology revealed a lymphohistiocytic infiltrate with histiocytes positive for CD 68 and S100 demonstrating emperipolesis (lymphocytophagocytosis) consistent with Rosai-Dorfman disease (figure). The patient had no CNS recurrence or systemic involvement after 12 months of follow-up.
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Figure Sagittal and axial T1-weighted MR images, hematoxylin and eosin-stained specimen, and S100-stained specimen
Sagittal (A) and axial (B) T1-weighted MR images revealing a contrast-enhancing extra-axial mass lesion with indentation of the cortex, dural thickening, underlying vasogenic edema, and a projection into an intersulcal region. Hematoxylin and eosin-stained specimen (C) shows a lymphohistiocytic infiltrate with histiocytes demonstrating emperipolesis (lymphocytophagocytosis). S100-stained specimen (D) shows histiocytes positive for S100.
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Rosai-Dorfman, sinus histiocytosis with massive lymphadenopathy, is a rare disease of the lymphoid tissues with systemic involvement in up to 25% of cases. Neurologic involvement is rare, occurring in 4% of patients in one series. There are approximately 80 cases of CNS involvement in the literature, and the majority of these cases are brain lesions presenting with focal deficits, seizures, or headaches due to mass effects.1 The typical lesions are dural-based with a predilection for the skull base. Isolated, intracranial Rosai-Dorfman disease can mimic a meningioma.2
Disclosure: The authors report no conflicts of interest.
Disclaimer: The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.
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REFERENCES
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- Kidd DP, Revesz T, Miller NR. Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology 2006;67:1551–1555.[Abstract/Free Full Text]
- Johnson MD, Powell SZ, Boyer PJ, Weil RJ, Moots PL. Dural lesions mimicking meningiomas. Hum Pathol 2002;33:1211–1226.[Medline]
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