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|Year : 2017 | Volume
| Issue : 1 | Page : 222-223
Choroid plexus involvement in Rosai–Dorfman disease
Bilal Battal, Salih Hamcan, Ugurcan Balyemez, Veysel Akgun
Department of Radiology, Gulhane Military Medical Academy, Ankara, Turkey
|Date of Web Publication||12-Jan-2017|
Department of Radiology, Gulhane Military Medical Academy, 06018, Etlik, Ankara
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Battal B, Hamcan S, Balyemez U, Akgun V. Choroid plexus involvement in Rosai–Dorfman disease. Neurol India 2017;65:222-3
Rosai–Dorfman disease (RDD) is characterized by an idiopathic non-neoplastic histiocytic proliferation in lymph nodes and extranodal areas. The disease is rarely encountered in neurological practice. RDD manifests as massive painless cervical lymphadenopathy, fever, and weight loss., Extranodal involvement is seen in approximately 43% of cases in the skin, orbit, respiratory tract and bones, and rarely in the central nervous system (CNS)., The radiological CNS manifestations of RDD resemble the appearance of a solitary or multiple en-plaque meningiomas. The choroid plexus involvement of RDD is an extremely rare entity that has been reported only in two cases.,
An 18-year-old male patient with proven RDD for 5 years was admitted to our hospital with headache and mild dizziness lasting for a month. According to the history, he had multiple cervical lymphadenopathy and also extranodal involvement of the facial skin, nasal mucosa, and soft tissue at other locations. There was no history of seizures. Physical and neurologic examination was unremarkable apart from several enlarged lymph nodes being palpated in the upper cervical chain. He had no visual field defect. Magnetic resonance imaging (MRI) of the brain showed bilateral enlargement, and diffuse and significant contrast enhancement, of the choroid plexus in the atria of both the lateral ventricles. In addition, there were areas of dural involvement observed in the tentorium, falx cerebri, and dural layer covering the planum sphenoidale that were characterized by asymmetric dural thickening, hypo- to isointense lesions on T1 and T2-weighted images and homogenous contrast enhancement, with neither edema nor mass effect [Figure 1].
|Figure 1: (a-d) contrast enhanced T1-weighted, and (e and f) T2-weighted magnetic resonance (MR) images show multiple extranodal dural involvement areas of the Rosai–Dorfman disease in the tentorium (white arrowheads), falx cerebri (thin white arrows), and dural layer covering the planum sphenoidale (thick white arrows) that is characterized with asymmetric dural thickening and homogenous contrast enhancement without edema or mass effect. MR images also reveal solid expansile lesions in the choroid plexus of both ventricular atria, characterized with hypo to isointensity on T2-weighted images and avid contrast enhancement on T1-weighted images (black asterisk)|
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Lymph node and extranodal involvement can be simultaneously seen in almost 43% of the cases with RDD whereas isolated extranodal involvement is seen in only 23% of the cases. In the reported cases with CNS involvement, 70% had no nodal disease and 52% had no associated systemic disease. Choroid plexus lesions due to RDD involvement are extremely rare and may mimic the appearance of papillomas, meningiomas, choroid plexitis, and granulomatous or inflammatory lesions.
Although intracranial involvement of the RDD is rare, it should be included in the differential diagnoses in cases with dural thickening and enhancement, as well as in the presence of enlarged choroid plexus with avid enhancement. In addition to the imaging techniques, histopathological and immunohistochemical evaluations are required for the definitive diagnosis.
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