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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary central nervous system atypical teratoid/rhabdoid tumors mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumor, medulloblastoma and choroid plexus carcinoma, but present different biological behaviors. We present the case of an 18 year-old man who presented headache, vomiting and ataxia. CT-scan and MRI revealed a posterior fossa tumor. A gross total resection was performed. An intraoperative study showed papillary-like tumors with large cells and mitotic features. Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells with papillary features and rhabdoid cells. Immunohistochemically, these rhabdoid cells were positive for vimentin, epithelial membrane antigen, smooth-muscle actin, cytokeratin, S-100 protein, and glial fibrillary acidic protein. Electro-microscopically, the typical rhabdoid cells contained whorled bundles of intermediate filaments in their cytoplasm. A rhabdoid tumor is a clinical-pathological entity and emphasizes the necessity to distinguish this unique tumor from other pediatric central nervous system neoplasms. Cytopathological features, immunohistochemistry and electro-microscopy differential diagnoses are discussed.
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PMID:Rhabdoid choroid plexus carcinoma: a rare histological type. 1787 20

Brain tumours form the most common type of solid tumour in children and more that 50% of these are infratentorial. Cerebellar astrocytomas and brain stem gliomas are the commonest posterior fossa glial tumours in children. Cerebellar astrocytomas represent up to 10% of all primary brain tumours and up to 25% of posterior fossa tumors in children, with Low grade gliomas forming the commonest of the cerebellar gliomas. They commonly present with symptoms and signs of raised intracranial pressure due to obstructive hydrocephalus. Radiologically they may be solid or cystic with or without a mural nodule. Surgical excision is the mainstay of treatment and forms the most consistent factor influencing progression free and long term survival. While majority of the tumours are pilocytic astrocytomas, they may also be fibrillary astrocytomas or even high grade tumours. Tumour histology does not appear to be an independent factor in the prognosis of these children, and therefore no palliative treatment after surgery is advocated. Brain stem gliomas account for approximately 10% of all pediatric brain tumours. Cranial nerve signs, ataxia and cerebellar signs with or without symptoms and signs of raised intracranial pressure are classically described symptoms and signs. Radiographic findings and clinical correlates can be used to categorize brain stem tumours into four types: diffuse, focal, exophytic and cervicomedullary. Histologically most brain stem gliomas are fibrillary astrocytomas. Diffuse brain stem gliomas are the most commonly seen tumour in the brain stem. These lesions are malignant high grade fibrillary astrocytomas. Focal tumours of the brain stem are demarcated lesions generally less than 2 cms in size, without associated edema. Most commonly seen in the midbrain or medulla, they form a heterogeneous pathological group, showing indolent growth except when the lesion is a PNET. Dorsally exophytic tumours lie in the fourth ventricle, while cervicomedullary lesions are similar to spinal intramedullary tumours. Expanding lesions are the only lesions amenable for excision while infiltrative and ventral lesions are not.
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PMID:Management of posterior fossa gliomas in children. 2206 33

A 6-year-old intact female Pointer dog was presented for evaluation of acute onset of ataxia, circling, and head tilt. Neurologic assessment revealed overall decreased postural reaction, left-sided hemiparesis with incoordination, rigidity of fore- and hindlimbs, strabismus of the right eye, and bilateral horizontal nystagmus. Using magnetic resonance imaging, a mass lesion was identified in the cerebrum adjacent to the left side of the cerebellum compressing the brain stem ventrally. The mass was incompletely resected, and during surgery fine-needle aspiration and biopsy of the mass were performed. Cytologically, smears were highly cellular and contained predominantly small to medium-sized discrete round cells with high nuclear to cytoplasmic ratios and round nuclei with rare deep clefts or indentation, smooth chromatin, and indistinct nucleoli. Numerous cytoplasmic fragments were noted in the background. The primary diagnosis was lymphoma; other differential diagnoses included neuroendocrine tumor and poorly differentiated tumor of neural origin. The histologic diagnosis was lymphoma, and the lesion was presumed to be metastatic. On immunohistochemical analysis, the cells expressed neither CD3 nor CD79a. Re-examination of the histologic section revealed disorganized sheets of cells with multifocal palisading and perivascular arrangements of rosette-like structures. An expanded panel of antibodies to vimentin, cytokeratin, glial fibrillary acid protein (GFAP), neuron-specific enolase (NSE), synaptophysin (SYN), S-100, and CD45 was applied to histologic sections. Neoplastic cells were immunoreactive for vimentin, NSE, and S-100. Based on the histologic appearance and immunophenotype of the tumor, a diagnosis of primitive neuroectodermal tumor (PNET) was made. PNET, although rare in dogs, should be considered as a differential diagnosis for round cell tumors in the brain.
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PMID:Cytologic and immunohistochemical characterization of a primitive neuroectodermal tumor in the brain of a dog. 2232 Jan 85

Neuroblastoma is the third most common malignancy of childhood, after leukemia and brain tumors. Only 2% of all neuroblastoma occur in the brain. Primary cerebellar neuroblastoma is an specific subset of Primitive Neuroectodermal Tumors (PNET). Meduloblastoma is a relatively common and well-established entity, consisting of primitive and multipotential cells that may exhibit some evidence of neuroblastic or gliad differentiation. But cerebellar neuroblastoma with ultrastractural evidence of significant neuroblastic differentiation is extremely rare. We report a rare case of neuroblastoma in the cerebellum. A 2.5-year-old Iranian boy presented with vomiting and nausea in the morning and ataxia. CT scan showed a tumor mass in the cerebellum and the report of radiologist was medulloblastoma. Light microscopic assay showed a small cell neoplasm with lobules of densely packed cells (lobulated pattern) and better differentiated cells. Neuron-Specific Enolase was positive. Pathologic diagnosis confirmed the existence of cerebellar neuroblastoma. Chemotherapy followed surgical removal. No relapse occurred 12 months after surgery.
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PMID:Cerebellar neuroblastoma in 2.5 years old child. 2525 Jan 30