Extranodal Rosai-Dorfman disease involving the meninges.

ABSTRACT

Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement has also been reported. We report the case of a 57-year-old woman presenting with seizures, focal motor deficits, and leptomeningeal enhancement on magnetic resonance imaging. Meningeal biopsy revealed Rosai-Dorfman disease. Sinus histiocytosis may mimic meningeal disease, and Rosai-Dorfman disease should be considered in the differential diagnosis of dural-based masses with diffuse leptomeningeal enhancement.

ROSAI-DORFMAN DISEASE was first described in 1969 as an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis with massive lymphadenopathy. (1) Cardinal features include massive, painless cervical adenopathy, fever, and polyclonal hypergammaglobulinemia. (2) Extranodal involvement has been reported in diverse anatomic sites, particularly the skin, orbit, and upper respiratory tract. (3,4) Central nervous system involvement without nodal disease has also been reported. (5,6) We describe the case of a 57-year-old woman who presented with seizures, focal motor deficits, and diffuse leptomeningeal enhancement on magnetic resonance imaging (MRI). Meningeal biopsy disclosed the histologic appearance of extranodal Rosai-Dorfman disease.

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CASE REPORT

A 57-year-old Caucasian woman presented to the hospital with transitory episodes of right upper extremity weakness, a 2-week history of frontoparietal headaches, and a single episode of tonic-clonic seizure. She denied arthralgias, skin rashes, fever, and weight loss. Her medical history was significant for severe asthma, which required treatment with inhaled steroids and salmeterol, and arterial hypertension treated with thiazides. Physical examination at the time of admission was remarkable only for mild weakness in the right upper extremity and mild bilateral wheezing on lung examination. No lymphadenopathy was palpated.

Laboratory values revealed a white blood cell count of 8.2 x [10.sup.3]/[micro]L; hemoglobin level of 12.7 g/dL, and platelet count of 384 x [10.sup.3]/[micro]L. Hypercoagulable state work-up disclosed normal levels of homocysteine, protein C, protein S, antithrombin III, and anticardiolipin antibodies. Total protein level was 6.7 g/dL and albumin level was 2.4 g/dL. Cerebrospinal fluid (CSF) examination showed normal levels of glucose and protein, and no red or white blood cells were found. Purified protein derivative (PPD) of tuberculin skin test, CSF tuberculosis DNA polymerase chain reaction (PCR), herpes simplex virus DNA PCR, and CSF cultures were all negative. Chest radiograph was negative for cardiomegaly, and there were no pulmonary infiltrates. Magnetic resonance imaging of the brain showed abnormal areas of leptomeningeal enhancement (Fig. 1). Differential diagnosis after imaging studies were completed included the possibility of infectious meningitis, leptomeningeal carcinomatosis, or meningioma. Due to the suspicion of neoplastic process, the patient underwent a meningeal biopsy. Frozen section material showed nodular aggregates of lymphoid cells, initially interpreted as malignant lymphoma. Permanent sections, however, demonstrated fibrotic dura that contained nodular and diffuse infiltrates of lymphoid cells admixed with aggregates of histiocytes and plasma cells. The histiocyte aggregates were immunopositive for the S-100 protein. All of these findings were consistent with extranodal Rosai-Dorfman disease (Fig. 2). The patient was treated with prednisone, and 3 months after diagnosis she remained seizure-free and without further symptoms.

DISCUSSION

Rosai-Dorfman disease is an uncommon disorder that typically manifests as systemic symptoms and lymphadenopathy in children and young adults. (1, 2) Extranodal intracranial disease is uncommon. The largest review of extranodal Rosai-Dorfman disease was by Andriko et al, (4) who described 11 cases of RosaiDorfman disease isolated to the central nervous system without nodal disease, and suggested the ability of this entity to mimic meningiomas, lymphomas, and chronic inflammation. In our patient, the initial clinical impression was that of cerebrovascular disease manifesting as transient ischemic events. The radiologic appearance suggested chronic meningitis, carcinomatous meningitis, or meningioma; the initial histopathologic impression was also that of a lymphoma. On more detailed examination, however, the lesions consisted of pale-staining histiocytes with emperiopolesis and lymphoplasmacytic infiltrates, characteristic of Rosai-Dorfman disease. In addition, immunohistochemical analysis of stains for S-100 p rotein were consistent with this condition.

CONCLUSION

The clinical presentation of our case suggests that sinus histiocytosis could manifest as meningeal disease, and could mimic meningioma or chronic meningitis. Rosai-Dorfman disease should be considered in the differential diagnosis of patients with dural-based masses and diffuse leptomeningeal enhancement on MRI.

References

(1.) Rosai J, Dorfman RF: Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity Arch Pathol 1969; 87:63-70

(2.) Foucar E, Rosai J, Dorfman R: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman) disease): a review of the entity Semin Diagn Pathol 1990; 7:19-73

(3.) Puppin D Jr, Chavaz P, Harms M: Histiocytic lymphophagocytic panniculitis (Rosai-Dorfman disease): a case report. Dermatology 1992; 184:317-320

(4.) Andriko JW, Morrison A., Colegial CH, et al: Rosai-Dorfman disease isolated to the central nervous system. A report of 11 cases. Mod Pathol 2001; 14:172-178

(5.) Woodcock RJ, Mandell JW, Lipper MH: Sinus histiocytosis (Rosai-Dorfman disease) of the suprasellar region: MR imaging findings--a case report. Radiology 1999; 213:808-810

(6.) Natarajan S, Post KD, Strauchen J, et al: Primary intracerebral Rosai-Dorfman disease: a case report. J Neurooncol 2000; 47:73-77

RELATED ARTICLE: KEY POINTS

* Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis with massive lymphadenopathy.

* Rosai-Dorfman disease usually manifests as massive cervical lymphadenopathy, fever, and polyclonal hypergammaglobulinemia.

* Extranodal Rosai-Dorfman disease has been reported to occur in diverse anatomic sites, including the skin, orbit, and upper respiratory tract; meningeal involvement has rarely been reported.

* Rosai-Dorfman disease manifesting in the meninges could mimic meningioma, lymphoma, or chronic meningitis.

From the Department of Medicine, Emory University School of Medicine, Atlanta, Ga.

Reprint requests to Carlos Franco-Paredes, MD, Department of Medicine, Emory University School of Medicine, 69 Butler St, Atlanta, GA 30303.