Article

Rosai-Dorfman disease mimicking multiple meningioma: case report.

Department of Pathology, Toronto Hospital, University of Toronto, Ontario, Canada.
Neurosurgery (impact factor: 2.79). 07/1995; 36(6):1185-7. pp.1185-7
Source: PubMed

ABSTRACT Rosai-Dorfman disease is a rare idiopathic histioproliferative disease affecting the lymph nodes. Extranodal involvement has also been recognized, but central nervous system manifestations are extremely rare. Only 12 patients with intracranial involvement have been reported previously, and they all presented with clinical and radiological findings suggestive of meningioma. We report multiple meningeal nodules in a patient presenting with seizures whose pathological findings at surgery confirmed the diagnosis of Rosai-Dorfman disease. A review of all previously reported intracranial lesions is presented.

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    Article: Stereotactic interstitial radiosurgery for intracranial Rosai-Dorfman disease
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    ABSTRACT: Background: Rosai-Dorfman disease is an idiopathic, histoproliferative disorder characterized by massive painless lymphadenopathy. The favorable treatment of Rosai-Dorfman disease affecting the central nervous system is surgical resection. Histological and immunohistochemical confirmation is essential for a definitive diagnosis. Case Report: The authors report on a 10-year-old patient with Rosai-Dorfman disease of the central nervous system who presented with increased intracranial pressure. She was treated by stereotactic interstitial irradiation using iodine-125 seeds (interstitial radiosurgery). Result: Stereotactic surgery was performed without complications. The patient recovered well to a normal neurologic status. MR images showed a complete remission 49 months after treatment. Conclusion: The presented case demonstrates the high efficacy and safety of interstitial irradiation for intracranial Rosai-Dorfman disease. Hence, interstitial radiosurgery could be an appropriate therapeutic option for high-risk resectable intracranial Rosai-Dorfman disease. Hintergrund: Das Rosai-Dorfman-Syndrom ist eine idiopathische, proliferative Erkrankung, welche durch eine schmerzlose Lymphknotenschwellung charakterisiert ist. Bei Befall des zentralen Nervensystems ist die mikrochirurgische Resektion die Therapie der ersten Wahl. Die histologische und immunhistochemische Analyse ist entscheidend für die definitive Diagnose. Fallbericht: Die Autoren berichten über eine 10-jährige Patientin mit erhöhten Hirndruckzeichen und einem zerebralen Rosai-Dorfman-Syndrom. Primär wurde eine stereotaktisch geführte interstitielle Brachytherapie mit Jod-125-Seeds durchgeführt. Ergebnis: Die Behandlung konnte ohne Komplikationen durchgeführt werden. Der neurologische Zustand normalisierte sich. MRT-Veraufskontrollen zeigten 49 Monate nach der Behandlung eine komplette Tumorremission. Schlussfolgerung: Der vorliegende Fall veranschaulicht die Effizienz der stereotaktisch geführten interstitiellen Seedbestrahlung zur Behandlung eines intrakraniellen Rosai-Dorfman-Syndroms und sollte insbesondere bei inoperablen Tumoren als Therapieoption berücksichtigt werden.
    Strahlentherapie und Onkologie 04/2012; 185(2):109-112. · 3.56 Impact Factor
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    Article: Case Report - Rosai-Dorfman disease mimicking a sphenoid wing meningioma
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    ABSTRACT: A 40-year-old male presented with a single generalized tonic-clonic seizure. MRI revealed an enhancing, dural-based, left lateral sphenoid wing lesion suggestive of a meningioma. At microsurgical excision, the lesion was firm and relatively avascular. The histopathological report revealed S-100 positive histiocytic proliferation with lymphophagocytosis (emperipolesis) characteristic of the Rosai-Dorfman disease. The case and its management are discussed.
    Neurology India (ISSN: 0028-3886) Vol 53 Num 1.

Keywords

12 patients
 
central nervous system manifestations
 
Extranodal involvement
 
intracranial involvement
 
intracranial lesions
 
lymph nodes
 
radiological findings suggestive
 
rare idiopathic histioproliferative disease
 
Rosai-Dorfman disease
 

M Kim