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).