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Diagnostic Slide SessionAmerican 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
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 rareolder patientsextra-axial mass HA, seizures, focal deficits
Radiologic DDx:
Hemangiopericytoma Gliosarcoma Leiomyosarcoma Hodgkin’s disease Plasmacytomas Neurosarcoidosis Plasma Cell Granuloma
Meningioma
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
References1. 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).
2. Theeler, B.J., Keylock, J.B. & Yoest, S.M. Teaching NeuroImage: isolated intracranial Rosai-Dorfman disease mimicking a meningioma. Neurology 70, e42 (2008).
3. 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).
4. 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).
5. Zhu, H., et al. Imaging characteristics of Rosai-Dorfman disease in the central nervous system. European journal of radiology 81, 1265-1272 (2012).
6. 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).
7. 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).