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

Division of Neurology, Nagoya University, Nagoya, Aichi, Japan
Journal of Neurosurgery (Impact Factor: 3.74). 05/2005; 102(4):733-7. DOI: 10.3171/jns.2005.102.4.0733
Source: PubMed


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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    ABSTRACT: Objective To enhance the understanding of Castleman’s disease (CD), and to improve its diagnosis and management. Methods Clinical features and related information on diagnosis and treatment of 14 cases of CD were retrospectively analyzed and the literature reviewed. Results Based on the clinical classification, localized CD was found in 8 of the 14 cases. Both the results of lymph node biopsy and histopathology indicated they were a hyaline-vascular type. The multicentric type CD was detected in 6 cases, among which 4 were plasma cell type and 2 mixed type based on histopathologic examination. There were a variety of clinical situations in the 14 cases, with a lack of specificity. They were previously misdiagnosed as other diseases, and final diagnosis depended on a histopathologic examination. The 8 patients with localized CD underwent excision, without recurrence up to now. The 6 patients with multicentric-type CD were treated with glucocorticoids or combined chemotherapy, and all achieved remission. Conclusions CD has complicated clinical manifestations and is difficult to diagnose. Lymph node biopsy is important for early diagnosis. An optimal curative effect can be achieved with a suitable therapeutic option, based on histopathology and clinical classification.
    No preview · Article · Jun 2007 · Chinese Journal of Clinical Oncology
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    ABSTRACT: Chordoid meningioma is an uncommon histopathological variant of meningioma with a peculiar chordoma-like appearance. Its association with systemic inflammatory disorder linked to Castleman's syndrome was confirmed in the majority of young patients, however such a relationship in adults remains enigmatic. We report two cases of chordoid meningiomas in adult patients without manifestation of Castleman's syndrome. One tumour was almost totally composed of chordoma-like areas whereas the second one exhibited the unique combination of chordoma- and chondroma-like pattern. This is the first description of chordoid meningioma combined with extensive cartilaginous metaplasia. Both tumours exhibited histological evidence of infiltrative growth, accompanied by a relatively high proliferative index within structures of chordoid appearance. The designation of the chordoid component in meningioma is very important as this subtype of meningioma exhibits a more aggressive biological behaviour and higher risk of recurrence.
    No preview · Article · Feb 2006 · Folia Neuropathologica
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    ABSTRACT: Chordoid meningioma (CM) is a meningioma containing regions that are histologically similar to chordoma, with trabeculae of eosinophilic, vacuolated cells in a myxoid background and corresponds to WHO grade II [1]. Such a tumor is a rare variant first described by Kepes et al. in 1988 in young patients associated with Castleman syndrome [2]. Only a series [3] and a few case reports [4-18, 24-30] were published. To the best of our knowledge, about 80 cases have been previously published, but unpublished cases are often recorded. Ultrastructural features of these tumors are studied in only few reports and are not yet precisely ascertained. We present a new case of a relapsing chordoid meningioma with aggressive behavior and acute clinical presentation and a "chordoid progression'' of the tumor characterized by histological and ultrastructural modification.
    Full-text · Article · Jul 2006 · Ultrastructural Pathology
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