Diagnostic Slide Session American Association of Neuropathologists

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Presentation transcript:

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

Disclosures No financial relationships to disclose

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

Imaging

Questions Additional special stains or studies? Diagnosis?

CD68

CD1a

S-100

S-100

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

Rosai-Dorfman Disease

Rosai-Dorfman Disease

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

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

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

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

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

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

References Johnson, M.D., Powell, S.Z., Boyer, P.J., Weil, R.J. & Moots, P.L. Dural lesions mimicking meningiomas. Human pathology 33, 1211-1226 (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, 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, Inc 14, 172-178 (2001). Zhu, H., et al. Imaging characteristics of Rosai-Dorfman disease in the central nervous system. European journal of radiology 81, 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 hematology 69, 67-71 (2002). Le Guenno, G., et al. Successful treatment with azathioprine of relapsing Rosai- Dorfman disease of the central nervous system. Journal of neurosurgery 117, 486- 489 (2012).