Diagnostic Slide Session American Association of Neuropathologists Caitlin

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?

CD 68

CD 1 a

S-100

S-100

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 • CD 68+, CD 1 a- , 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 1. 2. 3. 4. 5. 6. 7. 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 Neuro. Image: isolated intracranial Rosai-Dorfman disease mimicking a meningioma. Neurology 70, e 42 (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, 486489 (2012).