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J Neurosurg. 2005 Apr;102(4):733-7.

Intraventricular chordoid meningioma presenting with Castleman disease due to overproduction of interleukin-6. Case report.

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Department of Neurosurgery, Division of Pathology, Clinical Laboratory, Center for Genetic and Regenerative Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.


A rare case of chordoid meningioma in the lateral ventricle observed in an adult is reported. The first clinical manifestation of the disease was a prolonged fever of unknown origin. Abnormalities in the patient's blood chemistry, principally polyclonal hypergammaglobulinemia (immunoglobulin [Ig]G, IgA, and markedly IgE) and an elevated serum level of C-reactive protein, were associated with the disease. The tumor was histologically confirmed to be a chordoid meningioma, and its surgical removal resulted in complete resolution of the patient's symptoms. By combining reverse transcription-polymerase chain reaction and immunohistochemical analysis, it may be shown that cytokine production, including that of interleukin (IL)-6, IL-1beta, and vascular endothelial growth factor, plays a role in the pathogenesis of chordoid meningioma associated with Castleman syndrome.

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