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

Diagnostic Slide Session American Association of Neuropathologists Caitlin Latimer, M.D., Ph.D. University of Kentucky. No financial relationships to disclose. Disclosures. 15-year-old African-American male Prior “ eosinophilic cellulitis ” Several months of headaches  seizure.

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

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

  2. No financial relationships to disclose Disclosures

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

  4. Imaging

  5. Additional special stains or studies? Diagnosis? Questions

  6. CD68

  7. CD1a

  8. S-100

  9. S-100

  10. Final Diagnosis Rosai-Dorfman Disease of the dura

  11. Rosai-Dorfman Disease

  12. Rosai-Dorfman Disease

  13. 40% present with extranodal disease CNS involvement is rare older patients extra-axial mass HA, seizures, focal deficits Rosai-Dorfman Disease

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

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

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

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

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

  19. References • Johnson, M.D., Powell, S.Z., Boyer, P.J., Weil, R.J. & Moots, P.L. Dural lesions mimicking meningiomas. Human pathology33, 1211-1226 (2002). • Theeler, B.J., Keylock, J.B. & Yoest, S.M. Teaching NeuroImage: isolated intracranial Rosai-Dorfman disease mimicking a meningioma. Neurology70, 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 neuroradiology24, 515-518 (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, Inc14, 172-178 (2001). • Zhu, H., et al. Imaging characteristics of Rosai-Dorfman disease in the central nervous system. European journal of radiology81, 1265-1272 (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 hematology69, 67-71 (2002). • Le Guenno, G., et al. Successful treatment with azathioprine of relapsing Rosai-Dorfman disease of the central nervous system. Journal of neurosurgery117, 486-489 (2012).

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