Journal of Clinical Neuroscience 21 (2014) 1872–1873

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Journal of Clinical Neuroscience

journal homepage: www.elsevier .com/ locate/ jocn

Review

Dural-based Rosai–Dorfman disease: Differential diagnosticconsiderations

http://dx.doi.org/10.1016/j.jocn.2014.07.0110967-5868/� 2014 Elsevier Ltd. All rights reserved.

⇑ Corresponding author. Tel.: +1 216 444 8805; fax: +1 216 445 6967.E-mail address: praysor@ccf.org (R.A. Prayson).

Richard A. Prayson ⇑, J. Jordi RoweCleveland Clinic, Department of Pathology, L25, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA

a r t i c l e i n f o a b s t r a c t

Article history:Received 18 July 2014Accepted 28 July 2014

Keywords:Brain tumorDural massExtranodal sinus histiocytosis with massivelymphadenopathyRosai–Dorfman disease

Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) is a non-neoplastic condition that has rarely been reported to involve the central nervous system. This reportdocuments a 28-year-old man with Rosai–Dorfman disease who presented with a seizure and a dural-based mass that was thought to represent a meningioma. Resection showed a lesion marked by large,S-100 protein immunoreactive histiocytic cells with intermixed benign lymphocytes and plasma cells.Emperipolesis with intracytoplasmic lymphocytes and plasma cells was present. Differential diagnosticconsiderations will be discussed.

� 2014 Elsevier Ltd. All rights reserved.

1. Introduction

Extranodal sinus histiocytosis with massive lymphadenopathy(Rosai–Dorfman disease) is a rare benign condition of unknownetiology which typically causes cervical lymphadenopathy butcan involve other nodal and extranodal sites, with extranodalinvolvement reported in 43% of patients in one study [1,2].Systemic disease is often accompanied by fever, leukocytosis, apolyclonal hypergammaglobulinemia, and an elevated erythrocytesedimentation rate. Involvement of the central nervous system inthe absence of nodal disease is relatively rare. Many of these casespresent as a dural-based mass resembling meningioma on imagingstudies [3–5]. We present such a case in a 28-year-old man anddiscuss the pathologic differential diagnostic considerations.

2. Case report

The patient is a 29-year-old man who presented with a newonset seizure. The seizure prompted a MRI study which high-lighted an extra-axial 2.4 � 1.5 � 2.1 cm right posterior parietalconvexity lesion with a dural tail. The lesion was isointense tothe cortex on T1-weighted, T2-weighted and fluid attenuatedinversion recovery images and showed intense homogeneousenhancement on postcontrast studies. A radiographic diagnosis ofmeningioma was made. There was no evidence of abnormalities

or lymphadenopathy outside the central nervous system. Thepatient underwent resection of the lesion. Two months postopera-tively, he is seizure-free on antiepileptic medication.

Histologic sections showed a dural-based lesion marked byincreased numbers of large histiocytic cells intermixed with benignappearing lymphocytes and plasma cells (Fig. 1). Occasionalhistiocytes showed intracytoplasmic lymphocytes and plasma cellsconsistent with emperipolesis (Fig. 2). There was no evidence ofgranulomatous inflammation or necrosis. There was no evidenceof meningioma. Fite and Gomori methenamine silver stains failedto demonstrate microorganisms. Histiocytic cells demonstratedpositive staining with antibodies to CD68 (1:10 dilution; DAKO,Carpinteria, CA, USA) and S-100 protein (1:800 dilution; DAKO)(Fig. 3). CD1a immunoreactivity (prediluted; Serotec, Raleigh, NC,USA) consistent with Langerhans cell histiocytosis was notobserved.

3. Discussion

Most cases of neuroaxial involvement in Rosai–Dorfman diseasepresent as dural-based masses. Such a presentation conjures up adifferential diagnosis which includes a wide spectrum of disorders.The most common cause of a dural-based mass is meningioma.These tumors are marked by a proliferation of arachnoidal capcells. The rare lymphoplasmacyte-rich variant is marked by aprominent chronic inflammatory infiltrate but is usually devoidof the large histiocytes that mark Rosai–Dorfman disease.Epithelial membrane antigen immunoreactivity can be useful in

Fig. 1. The lesion was marked by large histiocytes with intermixed benignappearing lymphocytes and plasma cells (hematoxylin and eosin, original magni-fication � 200). This figure is available in colour at www.sciencedirect.com.

Fig. 3. The large histiocytic cells demonstrated S-100 protein immunoreactivity(original magnification � 200). This figure is available in colour atwww.sciencedirect.com.

Fig. 2. Scattered histiocytes with intracytoplasmic lymphocytes and plasma cellsrepresenting emperipolesis were present (hematoxylin and eosin, original magni-fication � 400). This figure is available in colour at www.sciencedirect.com.

R.A. Prayson, J.J. Rowe / Journal of Clinical Neuroscience 21 (2014) 1872–1873 1873

highlighting tumor cells in this setting. Other dural-based tumors,such as solitary fibrous tumor, hemangiopericytoma or metastaticcarcinoma have distinct morphologies that they are not likely to bepathologically confused with Rosai–Dorfman disease.

Infectious and inflammatory processes should be considered inthe differential. Mycobacterial infections and certain fungal infec-tions (for example, Cryptococcus) can have prominent numbers ofmacrophages in the meningeal region and present as inflammatorypseudotumors. Stains for microorganisms can be helpful in identi-fying the etiology. Sarcoidosis is characterized by granulomatousinflammation, not a typical feature of Rosai–Dorfman disease. A

rare entity, idiopathic hypertrophic pachymeningitis, can presentas a meningioma-like mass on imaging and is marked by a chronicinflammatory cell infiltrate, often times with a granulomatouscomponent, but lacks large, S-100 protein positive histiocytes.

Other histiocytic predominant processes which can mimicRosai–Dorfman disease include Langerhans cell histiocytosis whichis characterized by a mixed inflammatory infiltrate includingeosinophils and CD1a positive Langerhans cells with large, convo-luted nuclei; these cells may demonstrate S-100 protein positivity.Plasma cell granulomas are characterized by large numbers ofplasma cells admixed with lymphocytes and S-100 protein nega-tive histiocytes. Castleman’s disease, both hyaline vascular andplasma cell type, can rarely involve the central nervous system.Erdheim–Chester disease, a systemic histiocytosis, is marked byS-100 negative histiocytes and Touton multinucleated giant cells.

Conflicts of Interest/Disclosures

The authors declare that they have no financial or otherconflicts of interest in relation to this research and its publication.

References

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