1. Diagnostic Slide Session American Association of Neuropathologists
Caitlin Latimer, M.D., Ph.D.
University of Kentucky

2. Disclosures
No financial relationships to disclose

3. Case history 15-year-old African-American male
Prior “eosinophilic cellulitis”
Several months of headaches seizure

4. Imaging

9. Questions
Additional special stains or studies?
Diagnosis?

10. CD68

11. CD1a

12. S-100

13. S-100

14. Rosai-Dorfman Disease of the dura
Final Diagnosis
Rosai-Dorfman Disease of the dura

15. Rosai-Dorfman Disease

16. Rosai-Dorfman Disease

17. Rosai-Dorfman Disease
40% present with extranodal disease
CNS involvement is rare
older patients
extra-axial mass HA, seizures, focal deficits

18. Radiologic DDx: Meningioma Hemangiopericytoma Gliosarcoma
Leiomyosarcoma
Hodgkin’s disease
Plasmacytomas
Neurosarcoidosis
Plasma Cell Granuloma

19. Histopathologic DDx: Inflammatory myofibroblastic tumor
Infection/Abscess
Plasmacytoma
Langerhans Cell Histiocytosis

20. Rosai-Dorfman Disease: histology
Mixed inflammatory infiltrate
Large pale cells
CD68+, CD1a- , S-100+
Emperipolesis

21. Treatment & Prognosis Surgery, corticosteroids, radio/chemotherapy
Isolated CNS disease =
Widespread dissemination =

22. Follow-Up on Our Patient
Initial increase in residual tumor, but stable
Gradually tapered off Sz meds
Status epilepticus 1 yr ago
Suggested course of vinblastine and prednisone

23. References
Johnson, M.D., Powell, S.Z., Boyer, P.J., Weil, R.J. & Moots, P.L. Dural lesions mimicking meningiomas. Human pathology 33, (2002).
Theeler, B.J., Keylock, J.B. & Yoest, S.M. Teaching NeuroImage: isolated intracranial Rosai-Dorfman disease mimicking a meningioma. Neurology 70, e42 (2008).
Konishi, E., Ibayashi, N., Yamamoto, S. & Scheithauer, B.W. Isolated intracranial Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). AJNR. American journal of neuroradiology 24, (2003).
Andriko, J.A., Morrison, A., Colegial, C.H., Davis, B.J. & Jones, R.V. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 14, (2001).
Zhu, H., et al. Imaging characteristics of Rosai-Dorfman disease in the central nervous system. European journal of radiology 81, (2012).
Pulsoni, A., et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. American journal of hematology 69, (2002).
Le Guenno, G., et al. Successful treatment with azathioprine of relapsing Rosai- Dorfman disease of the central nervous system. Journal of neurosurgery 117, (2012).