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

Department of Neurosurgery, Division of Pathology, Clinical Laboratory, Center for Genetic and Regenerative Medicine, Nagoya University Graduate School of Medicine, 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: ObjectiveTo enhance the understanding of Castleman’s disease (CD), and to improve its diagnosis and management. MethodsClinical features and related information on diagnosis and treatment of 14 cases of CD were retrospectively analyzed and the literature reviewed. ResultsBased 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. ConclusionsCD 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.
    Chinese Journal of Clinical Oncology 06/2007; 4(3):195-200. DOI:10.1007/s11805-007-0195-4
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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.
    Ultrastructural Pathology 07/2006; 30(4):309-14. DOI:10.1080/01913120600820591 · 1.08 Impact Factor
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    ABSTRACT: The study has been undertaken to document the clinicopathological features of 12 cases of chordoid meningioma, operated at All India Institute of Medical Sciences during 1996 to June 2005. Clinical information was retrieved from the records of our Neurosurgery Department. The cases were stained with H&E, Periodic Acid Schiff (PAS) with and without diastase, mucicarmine, giemsa, toluidine blue, alcian blue, reticulin and Masson trichrome. Immunohistochemistry for pancytokeratin, epithelial membrane antigen, vimentin, glial fibrillary acidic protein, MIB-1, Leucocyte common antigen (LCA), CD-3 and CD-20 was done in all cases. The age ranged from 12-67 years (mean 34.2 years) and three of them occurred in < 18 years. Male to female ratio was 1:1.4. The duration of symptoms varied from 3.5 months to 5 years (mean 14.1 months). No systemic symptoms were noted. The location of tumor in eight cases was in the supratentorial and rest four in the infratentorial compartments. Interestingly, two cases were in intraventricular location, one in the lateral ventricle and other in the fourth ventricle. Microscopic examination showed lobulation with chordoid elements constituting > 95% of the entire tumor area in 11 of the total 12 cases. In one case, chordoid pattern constituted about 30% of the total tumor area; the rest was predominant meningothelial (60%). Mild to severe lymphoplasmacytic cell infiltrate was present in all cases. The histochemical stains showed the pattern of acidic mucin and interestingly revealed the presence of mast cells both in connective tissue stroma and epithelial cell islands. The inflammatory infiltrate was B-cell predominant. MIB-1 labeling index was low (< 2%) in all cases except two, which showed LI of 6% and 8%. Strong diffuse immunoreactivity for vimentin and focal positivity for epithelial membrane antigen was noted in all cases. Chordoid meningiomas are predominantly tumors of young adults with predilection for supratentorial location. Intraventricular location, absence of systemic manifestations despite the presence of abundant B-lymphocytes, presence of mast cells and low MIB-1 LI are some of the interesting findings in the present series, which need documentation. Hence, larger number of cases with adequate follow-up data need to be studied further to establish the clinical significance of this variant.
    Journal of Neuro-Oncology 08/2006; 78(3):263-9. DOI:10.1007/s11060-005-9092-y · 3.07 Impact Factor
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