bromochloroacetic-acid and Neurofibromatosis-2

Clear cell meningioma (CCM) is a rare variant of meningioma, which is important to distinguish because of its aggressive behaviour. Sixty-eight cases have been previously described in the literature. In this retrospective study, we report seven cases of CCM operated in our institution between 1994 and 2008.. Seven CCM cases were retrieved from the files of our pathology department. Clinical and radiological data were reviewed. A standard histological study was realized and immunohistochemistry was performed with epithelial membrane antigen (EMA), cytokeratin KL1, progesterone receptors, Ki-67 (MIB-1), S100 protein.. Patients' age ranged from 2 to 70 years (median age: 36 years), with a female predominance (5/7 patients). Three patients belonged to the same family, probably affected by neurofibromatosis type 2. CCM occurred in various locations: medullary region (two), sphenoid wing (two), ponto-cerebellar angle (two), tentorium (one). The tumour could be fully resected in three cases. Follow-up ranged from 3 months to 15 years: recurrence occurred in four patients, three of whom eventually died from the disease.. In our series, the frequency of CCM (0,6% of all meningiomas operated on in our institution) and its histological aspects are almost identical to those observed of the literature. We discuss the predictive value of proliferation index (MIB-1) and the role of patient status and quality of surgical resection in the evolution.. Our study supports the fact that MCC course is less favourable than meningioma WHO grade I, even in the absence of anaplastic area, high mitotic activity, or necrosis. In this series, MIB-1 index was of no interest identifying patients with or without recurrence.

Topics: Adult; Aged; Astrocytoma; Biomarkers, Tumor; Child, Preschool; Diagnostic Errors; Ependymoma; Female; Humans; Keratins; Ki-67 Antigen; Male; Meningeal Neoplasms; Meningioma; Middle Aged; Mucin-1; Neoplasm Proteins; Neurofibromatosis 2; Receptors, Progesterone; Retrospective Studies; S100 Proteins; Young Adult

To gain insight into the pathogenesis of neurofibromatosis type 2 (NF2) by investigating the ocular manifestations of this disease.. Using standard histologic techniques, immunohistochemistry, and electron microscopy, we described the ocular pathologic findings of a 34-year-old woman who died from complications of NF2.. We identified 3 types of NF2-associated lesions: juvenile posterior subcapsular cataracts, epiretinal membranes, and an intrascleral schwannoma.. Our analysis indicated that dysplastic lens cells accumulate just anterior to the posterior lens capsule in juvenile posterior subcapsular cataracts and that dysplastic Müller cells may be a major component of NF2-associated epiretinal membranes. Clinical Relevance Our findings suggest that a subset of glial cells with epithelial features (Schwann cells, ependymal cells, and Müller cells) may be particularly sensitive to loss of the NF2 gene. Understanding the molecular basis for this sensitivity may lead to novel strategies for treating NF2.

Topics: Adult; Cataract; Epiretinal Membrane; Eye Neoplasms; Fatal Outcome; Female; Glial Fibrillary Acidic Protein; Humans; Immunoenzyme Techniques; Keratins; Mucin-1; Neurilemmoma; Neurofibromatosis 2; S100 Proteins; Scleral Diseases