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Neurology India, Vol. 59, No. 3, May-June, 2011, pp. 329-330 Editorial Rosai-Dorfman Disease and neurological manifestations C Sundaram Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India Correspondence Address: C Sundaram Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh India challa_sundaram@yahoo.com Date of Submission: 24-May-2011 Code Number: ni11103 PMID: 21743156 DOI: 10.4103/0028-3886.82705 Rosai-Dorfman Disease (RDD), sinus histocytosis with massive lymphadenopathy is an idiopathic histocytic proliferation affecting the lymph nodes in its classic form. It may affect many diverse sites with or without nodal involvement; the estimated incidence of classic nodal presentation of RDD is approximately 100 cases per year. The extranodal disease is seen in about 43% of cases and central nervous system (CNS) involvement is seen in less than 5% of cases. [1],[2] A review of the literature on CNS involvement in RDD from 1969-2008 observed a sudden increase in the reports of RDD of CNS from 2002-2008. [3] Review of Indian literature between 1996 and 2010 revealed report of 20 cases of RDD of CNS, mostly from south India, 17 cases. [4],[5],[6],[7],[8],[9],[10],[11],[12] This included 18 patients with intracranial lesions, one patient with spinal, and one patient with both intracranial and spinal lesions. There were two patients with multiple intraparenchymal lesions and one patient with dural-based lesions involving multiple levels. The intracranial dural-based lesion involved bone in two patients and there was erosion of bone in two patients. All the cases of RDD were isolated involvement of CNS and confirmation of diagnosis was made by histopathology and immunohistochemistry. None of the patients were diagnosed preoperatively. [4],[5],[6],[7],[8],[9],[10],[11],[12] CNS involvement in RDD affects all the age groups with a mean age of 39.3 years. [3] About 70% patients with intracranial RDD had no lymphadenopathy and 52% had no systemic disease [3],[12] In this issue of the journal, Venkidesh et al., [13] report a patient with isolated intracranial RDD without nodal involvement. Intracranial lesions were reported in 77%, spinal lesions in 14% and both intracranial and spinal lesions in 9% of patients. [3],[14],[15] Majority (85%) of the intracranial lesions were dural-based lesions mimicking meningioma located in the convexity or skull base. Intraparenchymal lesions are rarely reported. The spinal lesions may be cervical, thoracic or lumbar. [3] Maiti et al., [16] has reported a patient with spinal extradural lesion in this issue of the journal. Intramedullary involvement is rare. Lesions may be single or multiple. There may be invasion of the dura, bone or sinuses. Multiple intraparenchymal lesions are extremely rare. [5],[7] Neuroimaging features are often non-specific and a preoperative diagnosis is often difficult. On computed tomography (CT), the lesion may appear as a homogenous lobulated hyperattenuating mass with marked contrast enhancement. There may be moderate to marked perilesional edema with mass effect. There can be associated bone erosion but not calcification. [14],[15] On magnetic resonance imaging (MRI), the lesions are lobulated, isointense on T1W1 with homogenous intense enhancement on gadolinium injection. On T2W1 the lesions appear hypo- to isointense. MR spectroscopy with elevated choline may improve the specificity of preoperative diagnosis and help in differential diagnosis from meningioma. [17] Histopathology forms the mainstay of diagnosis. Classically, the lesions are composed of lymphoplasmacytic infiltrate and scattered Russel bodies. [1],[2] There is characteristic histiocytic proliferation and emperipolesis. Though the features are essentially similar to nodal disease, emperipolesis is less pronounced and fibrosis is more in extranodal disease. Immunohistochemistry demonstrates positivity for CD 68 and S100 due to the histiocytic nature of the lesion and RDD needs to be differentiated from other inflammatory and neoplastic lesions of the CNS, especially dural-based lesions. These include Langerhans cell histiocytosis, Hodgkin's lymphoma, plasmacytoma, plasma cell granuloma or inflammatory pseudotumor. [18] The emperipolesis and immunohistochemical evaluation will help to rule out other diagnostic possibilities, especially in the isolated form where there is no lymphadenopathy or other system involvement. The etiology of RDD is still uncertain. Molecular studies have demonstrated that RDD is a polyclonal disorder. A definite infectious agent has not been isolated though various studies have been carried out to isolate an etiological agent. In conclusion CNS involvement in RDD is still rare, though there is an increase in the documentation of CNS-RDD. However, because of the ubiquity of its involvement of the entire neuraxis and its ability to mimic meningeal and primary brain tumors, it is essential to be aware of this entity and consider RDD in the differential diagnosis of various lesions of the CNS. References
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