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Hypertrophic pachymeningitis is extremely rare. It is a fibrosing inflammatory process which involves the dura mater, including the tentorium. Numerous pathological entities produce thickening of the pachymeninges, so that idiopathic hypertrophic pachymeningitis is a diagnosis of exclusion. We describe four patients with idiopathic hypertrophic pachymeningitis who had varied clinical presentation. Imaging studies revealed diffuse thickening of the pachymeninges; in one patient there was extensive dural sinus thrombosis. Since no identifiable cause was found, the cases were labelled as idiopathic.

Most central neurocytomas (CN) and spinal neurocytomas (SN) have a bland well-differentiated histologic picture and uneventful clinical course. However, rare examples showing histologic atypia, recurrence and even CSF dissemination have been reported. Herein we report a case of recurrent spinal neurocytoma in a 24-year-old male who presented with a 2-month history of weakness and numbness of the left upper and lower limbs, and was previously operated at the same site 10 months ago. MRI revealed a contrast enhancing intramedullary mass involving C5-T1 region. Radiologic and operative impression at both surgeries was that of a glioma, possibly anaplastic. Histologic and immunohistochemical features in both resections were those of an atypical neurocytoma. The tumor showed rare mitoses, focal mild vascular proliferation in both specimens, and necrosis in the initial specimen. MIB1 labeling indices were 9 and 10%, respectively. Based on the analysis of this case and limited data from the literature, it is hypothesized that SN shows a histopathologic picture, immunoprofile and biologic behavior very similar to CN. However, the presence of histologic atypia and increased MIB1 index in SN appear to more closely correlate with tumor recurrence and a worse overall outcome, in part due to their location in the critical region of cervical spinal cord. Therefore, we hypothesize that SN with atypia requires a close clinical follow up. As in CN, radiation therapy is perhaps best reserved for atypical, progressive and recurrent SN.

Dysembryoplastic neuroepithelial tumor (DNT) is a relatively newly described entity and is an important cause of intractable epilepsy. We report 32 cases of DNT who were operated and treated in our hospital over a period of 12 years. Immunostaining for various proliferative markers and tumor suppressor gene proteins was done to assess the proliferative potential of these tumors. The most common presentation was partial complex seizures followed by generalized tonic-clonic seizures, focal motor seizures, and myoclonus. The most common location was temporal lobe followed by frontal and in one patient lesion was multifocal. All patients were seizure free at the last postoperative follow-up which varies from 12 to 96 months with mean of 33.7 months. Microscopic examination showed classical histology comprising of intracortical multinodular microcystic lesions with floating neurons. Proliferative indices were very low (<1%) and tumor suppressor gene protein expression was not seen in the present study. Cortical dysplasia of the surrounding brain was observed in 37.3% of cases.

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Intracranial dermoid and epidermoid cysts are usually considered to be two different entities in the radiological and surgical literature. Epidermoid cysts are classically off midline in location, isointense to cerebrospinal fluid on T1 and T2-weighted images and have restricted diffusion, whereas dermoid cysts are classically midline in location, have T1-hyperintense regions due to the presence of fat and show facilitated diffusion. We report a case of radiological epidermoid cyst in baseline imaging, which evolved into a radiological dermoid cyst over time, and explain this unique occurrence with a review of the embryology and histopathogenesis of these cysts.