Chordoid meningioma is a rare meningothelial tumor characterized by chordoma-like histological features with lymphoplasmacellular infiltration. This tumor is often seen in children, but not in adults, with a systemic inflammatory syndrome (iron-resistant microcytic anemia and/or dysgammaglobulinemia) and very rarely with a persistent moderate hyperthermia. In the present report the authors describe a temporal chordoid meningioma in a 30-year-old woman who presented with fever, headache, and a serological inflammatory syndrome. The clinical symptomatology, chiefly the fever, disappeared immediately after removal of the tumor. To the authors' knowledge, only one similar patient with such clinical presentation and response to surgery has been mentioned in the literature. Interestingly, at immunohistochemical examination, the neoplasm showed focal positivity for the pyrogenic cytokine interleukin-6. The capacity of the tumor to produce this pyrogenic cytokine could explain both the patient's clinical presentation and her response to the surgical management.
[Show abstract][Hide abstract] 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 . Such a tumor is a rare variant first described by Kepes et al. in 1988 in young patients associated with Castleman syndrome . Only a series  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 To the best of our knowledge, about 80 cases have been previously published, but unpublished cases are often recorded.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.
[Show abstract][Hide abstract] ABSTRACT: Intraventricular meningiomas are infrequent intracranial tumors. Clinical symptoms are mainly due to an increased intracranial pressure or a direct pressure on the surrounding brain structures. Inflammatory syndrome was described in some patients with chordoid meningiomas. Here we report a case of right intraventricular clear cell meningioma in a 50-year-old man who presented with fever, headache, and inflammatory syndrome. Clinical and biological normalization was rapidly obtained after tumor removal. Immunohistochemical examination showed tumor cells and lymphocytes positivity for the pyrogenic cytokine interleukin-6, with a same intensity. To our knowledge, this is the first case described in the literature concerning an adult man with an intraventricular clear cell meningioma associated with a systemic inflammatory syndrome.
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