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1/4. Dysembryoplastic neuroepithelial tumor. A case report.

    Dysembryoplastic neuroepithelial tumor (DNT) is a rare, benign tumor encountered in the cortex. It is characterized by the presence of cells of different histogenesis. Due to its mixed nature (glial-neuronal), WHO histological classification of brain tumors included it into the group of neuronal and glial-neuronal mixed tumors. Case of tumor in a 19-year-old woman experiencing for three years seizure of temporal lobe epilepsy is presented. A cranial magnetic resonance imaging (MRI) showed "pseudocystic" tumor in temporal lobe. Histological and immunocytochemical examinations of the tumor fragment removed during surgery revealed large numbers of neuronalglial nodules occurring in the cerebral cortex. Columns of glial-neuronal structures crossing parallely to the cortex surface, surrounded by oligodendrocyte-like cells (OLC) were a characteristic feature of the tumor texture. In the tumor interstitium, "floating" maturated, dysplastic-free ganglionic cells were visible in numerous bright spaces. In addition, numerous lobuliform--structured areas consisted of oligodendrocyte-like cells. Oligodendrocyte-like cells were characterized by positive immunoreaction to the presence of S-100 protein and synaptophysin. Basing on clinical manifestation and histopathological findings dysembryoplastic neuroepithelial tumor was diagnosed.
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2/4. Gliomatosis cerebri: disconnection of the cortical grey matter, demonstrated on PET scan.

    18-FDG and 11C methionine PET scans were performed on two patients with gliomatosis cerebri. The cortical grey matter was hypometabolic when compared with normal. The findings support the concept that the cerebral cortex becomes functionally disconnected in this disease owing to the infiltrative nature of the underlying tumour. This may account for the high incidence of dementia in the course of this disease. In one of the cases described here, there was clear evidence of progression from a discrete tumour mass of glioma to gliomatosis cerebri and this progression argues against the WHO classification of this disorder separately from glioma.
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3/4. Dysplastic glioneuronal lesion arising in the cerebellum: a clinicopathological, immunohistochemical and ultrastructural study.

    We describe a case of dysplastic glioneuronal lesion in the right cerebellar hemisphere. A 13-year-old boy presented with headache since 1998. He had no neurological deficits. The computerized tomograph (CT) scan showed prominent calcification, and magnetic resonance imaging (MRI) revealed a non-enhancing mass of 15 x 15 x 5 cm in the right cerebellar hemisphere. The mass had low intensity in T1- and high intensity in T2-weighted images. Histologically, the lesion was composed of poorly defined small to intermediate sized cells arranged in fibrillar background. Although few neuronal cells having large nuclei with small nucleoli were present, no ganglion cells could be seen. Immunohistochemically, these poorly defined cells were non-reactive to various glial and neuronal markers. However, GFAP, synaptophysin, neurofilament and vimentin-reactive intercellular matrix and few nonneoplastic GFAP-positive glial cells and neurofilament-positive neuronal cells were seen. A very low MIB-1-labelling index of less than 0.1% was noted. Ultrastructurally, two different populations of the cells were seen. A few neuronal cells were larger and had an oval nucleus with small nucleolus and cytoplasm containing various cytoplasmic organelles, golgi apparatus, mitochondria, ribosomes, lipofuscin, rough endoplasmic reticulum, microtubules and neurofilaments. Many other cells had a scant cytoplasm and thus poorly defined. Cytoplasmic processes with axono-dendritic synapses and foci of bundles of intermediate filaments were present in the intercellular areas of the lesion. Based on these radiological, histological and ultrastructural findings of the lesion of low proliferative potential, we considered it dysplastic in nature.
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4/4. Papillary neuroepithelial tumor of the pineal region. A case report.

    We present here an unusual case of papillary neuroepithelial tumor of the pineal region. The patient was a 29-year-old female who presented with headaches. A computed tomography scan revealed a tumorous lesion at the pineal region and hydrocephalus. The resected tumor was composed of columnar and cuboidal cells showing characteristics of papillary growth. The tumor cells exhibited diffuse and intense immunoreactivity to cytokeratins and neural cell adhesion molecule. The tumor expressed abundant levels of transthyretin (prealbumin) and appeared ependymal in nature, with numerous microlumens delineated by punctate and ring-like patterns in epithelial membrane antigen staining. Reactivity to synaptophysin and glial fibrillary acidic protein was observed only in the infiltrated non-neoplastic pineal parenchyma. These histological characteristics matched the description of the recently reported papillary tumor of the pineal region thought to originate from the specialized ependyma of the subcommissural organ (SCO). Transthyretin expression of the present case further supports the likelihood of SCO origin, as transthyretin is one of the proteins presumed to be secreted by human SCO.
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