Cases reported "Brain Neoplasms"

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1/23. Benign isolated fibrohistiocytic tumor arising from the central nervous system. Considerations about two cases.

    Benign fibrous histiocytomas (BFHs) are tumors with fibroblastic and histiocytic components without histological anaplasia. Intracerebral lesions are exceptional and to our knowledge a spinal location was not yet described. We describe 2 cases of BFHs of the neural axis: the first, a 22-month-old boy with Down's syndrome, presented with a paraparesis and the magnetic resonance (MR) of the spine disclosed an intradural extramedullary, thoracic mass, totally resected; the second, a 13-year-old boy with left partial motor seizures, in whom the MR of the brain showed an intracerebral, right frontal tumor, also surgically removed. Both patients are free of recurrence, 6 years and 15 months after surgery, respectively. Histological examination and immunoreactivity for vimentin and histiocytic markers favored the diagnosis of BFH. It is likely that these tumors may originate from spinal dura mater mesenchymal stem cells and from the intracerebral perivascular pial sheath or the brain vessel walls themselves, respectively. Other benign, isolated, intracranial fibrohistiocytic neoplasms, namely the juvenile xanthogranuloma, can harbor a clinical, morphological and immunohistochemical profile overlapping the one of the BFH. Intracranial germ cell tumors may be associated with Down's syndrome, although harboring an unusual, non-pineal and non-chiasmatic location. One can speculate that a similar, still unknown genetic mechanism responsible for this association, could also induce the growth of other type of tumors in patients with this syndrome. BFHs should be added to the differential diagnosis of intracerebral or spinal dural attached tumors. Furthermore, we propose to name these intracranial tumors "benign isolated fibrohistiocytic tumors of the CNS".
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2/23. Gliofibrous nodule in the cerebello-medullary fissure.

    An extra-axial nodule in the cerebello-medullary fissure is described, occurring in a 27-year-old-woman. MRI and CT scans revealed the lesion was a non-enhanced round mass, which was associated with mild atrophy of the surrounding cerebellum, but with no perifocal edema. In the surgical observation, the mass was white, elastic and hard, well demarcated and localized in the cerebello-medullary fissure. Histologically, the lesion was composed of astrocytes and collagen-producing fibroblasts with no anaplasia. These findings suggested that the lesion was hamartomatous, but not neoplastic. This type of gliofibrous nodule has not been previously reported.
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3/23. Molecular genetic analysis of anaplastic pleomorphic xanthoastrocytoma.

    A case of pleomorphic xanthoastrocytoma in a 10-year-old Malay boy is reported. The patient presented with headache and epilepsy. On computed tomography, a ring-enhancing low-density lesion was observed in the left fronto-temporal area. During surgery, a cystic tumour containing serous fluid was found and almost totally removed. Histologically, the tumour exhibited marked pleomorphism of oval and spindle-shaped cells intermixed with uni- and multinucleated giant cells, and xanthomatous cells with foamy cytoplasm. The tumour displayed pericellular reticulin and periodic acid-Schiff positive granules. Focally, six mitotic characters per 10 high-power fields were seen, and necrosis was confined only to the inner lining of the cyst. Mutational analysis showed that a frameshift mutation (a 4-bp deletion) in the p53 gene had occurred in codons 273 and 274 of exon 8. No mutation was detected in the p16 gene. No allelic loss and/or loss of heterozygosity were observed on chromosome 10 using microsatellite marker D105532. The patient was treated with postoperative radiotherapy because of histological anaplasia and the presence of residual tumour. The patient showed marked neurological recovery after a follow-up period of 2 years.
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4/23. Meningeal melanocytoma: an aggressive course for a benign tumor.

    A 79-year-old female presented with difficulty ambulating and was found to have weakness and hyperreflexia in the lower extremities. magnetic resonance imaging (MRI) revealed a large T8-T9 intraspinal tumor. She underwent a thoracic laminectomy, and excision of an intradural extramedullary lesion. The surgical specimen was soft, black tissue that consisted of a moderately cellular, deeply pigmented tumor. The neoplastic cells proved to be melanocytic, and were devoid of overt features of anaplasia, i.e., prominent nuclear pleomorphism, necrosis, significant mitotic activity, and high proliferation indices. Four months postoperatively, MRI demonstrated focal areas of enhancement in the conus medullaris and in the fourth ventricle, indicating leptomeningeal spread. Subsequently, the patient underwent whole brain radiation. On repeat imaging, there was nodular enhancement of the fourth ventricle and throughout the spinal cord. Despite chemotherapy and radiation therapy, the disease advanced and the patient expired. Meningeal melanocytoma is a rare, histologically benign tumor with good prognosis. However, local aggressive behavior has been recorded, especially in cases of subtotal gross resection. On a literature review, there was one case of cranial posterior fossa meningeal melanocytoma with associated lesions in both suprarenal glands and the left kidney, but there were no cases with distant metastasis. In this report, we present an unusual case of spinal meningeal melanocytoma with diffuse spread throughout the craniospinal axis that proved to be fatal.
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5/23. ependymoma with neuropil-like islands: a case report with diagnostic and histogenetic implications.

    We describe a case of ependymoma with neuronal differentiation in form of neuropil-like islands. A 6-year-old boy presented at clinical examination for a short history of headaches and vomiting. brain computed tomography showed a large, partially cystic, parieto-occipital lesion. The tumor was composed by glial fibrillary acidic protein-positive round cells with a perivascular arrangement and scattered neuropil-like islands, showing intense positivity for synaptophysin. Despite radiotherapy, the tumor recurred, showing frank features of anaplasia, but lacking the neuropil-like islands. The histological features of the tumor are discussed in the light of the concept that neuronal differentiation can occur occasionally in gliomas of different lineage without affecting the expected biological behavior.
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6/23. Papillary glioneuronal tumor.

    The descriptive term papillary glioneuronal tumor (PGNT) has been repeatedly applied to a morphologic subset of low-grade mixed glial-neuronal neoplasia of juvenile and young adult patients. We report on a 13-year-old boy with PGNT of the left temporal lobe, who presented with headaches and a single generalized seizure. On magnetic resonance imaging, tumor was seen as a large, moderately enhancing paraventricular mass with cyst-mural nodule configuration and slight midline shift. Perifocal edema was virtually absent. Gross total resection could be performed, followed by an uneventful recovery. Histologically, the tumor exhibited similar, if not identical, features as reported previously. These comprised a patterned biphasic mixture of sheets of synaptophysin-expressing small round cells and pseudorosettes of GFAP-positive rudimentary astrocytes along vascular cores. Focally, the latter imprinted a pseudopapillary aspect on this otherwise solid lesion. Both cellular components expressed non-polysialylated neural cell adhesion molecule (NCAM)-L species, and several overlapping areas of synaptophysin and GFAP immunoreactivity were present. The mean MIB-1 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation, or necrosis were consistently lacking. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular neurocytic neoplasia, whose paradigmatic presentation may allow for consideration as an entity.
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7/23. Desmoplastic infantile ganglioglioma with high proliferation index: report of a case.

    Desmoplastic infantile ganglioglioma (DIG) is an uncommon neuroepithelial tumor associated with epilepsy, mostly occurring in the first 2 years of life. Most DIGs carry good prognosis after complete resection, even when a primitive cellular element is present. However a few examples of DIG with histologic anaplasia have recently been reported, and one demonstrated an unusual aggressive behavior. The authors describe herein a DIG with high Ki-67 proliferation index (30%) in a 10-month-old male infant with epilepsy, but with an excellent prognosis after total tumor resection.
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8/23. Pediatric cerebellar pleomorphic xanthoastrocytoma with anaplastic features: a case of long-term survival after multimodality therapy.

    CASE REPORT: A 4-year-old girl had a large midline cerebellar solid and cystic mass partially attached to the meninges. The original diagnosis was glioblastoma multiforme and she was treated by a gross-total surgical resection followed by chemotherapy and radiation therapy to the posterior fossa during the ensuing 14 months. She has received no further therapy and appears to be doing well 12 years later. This unusual favorable clinical outcome prompted our review of this case. methods: Additional special stains and immunocytochemistry were performed on the paraffin embedded tumor sections. RESULTS: We have confirmed the original histopathological observations of hypercellularity and focal nuclear pleomorphism, atypical mitoses, vascular hyperplasia, as well as focal necrosis. However, the additional stains revealed that the tumor is a relatively well-circumscribed meningeal-based astrocytic tumor (positive for GFAP) with extensive reticulin deposit and focal neuronal differentiation (positive for synaptophysin). A Ki67 labeling index is generally very low, but is positive in up to 5-10% of tumor cells focally. In the light of the favorable clinical outcome and the overall histological features, this tumor may be best reclassified as a rare example of cerebellar pleomorphic xanthoastrocytoma with foci of anaplasia.
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9/23. Regression of grade III astrocytoma during the treatment of CML with imatinib mesylate.

    Astrocytomas are central nervous system neoplasms, which are derived predominately from astrocytes. On the basis of the histopathologic characteristics astrocytomas are graded from I to IV. The cells that demonstrate the greatest degree of anaplasia are used to determine the histologic grade of the tumor. The mean age of survival are approximately 10 years from the time of diagnosis for pilocystic astrocytomas (world health organization grade I), more than 5 years for patients with low-grade diffuse astrocytomas (WHO grade II), 2 to 5 years for those with anaplastic astrocytomas (WHO grade III), and less than 1 year for patients with glioblastoma (WHO grade IV). The treatment is a combination of surgery, radiation, and chemotherapy depending of the grade of astrocytoma. We present a case of 31-year-old man with grade III astrocytoma with subsequent chronic myelogenous leukemia treated with imatinib mesylate as part of his chronic myelogenous leukemia treatment failing to show recurrence of the astrocytoma 10 years after standard treatment for astrocytoma.
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10/23. Intracranial astrocytoma: pathology, diagnosis and clinical presentation.

    Approximately 15,000 deaths a year occur in the united states from brain tumors. Astrocytomas, the most common primary tumor of the central nervous system, develop from astrocytes, the neuroglial support cell. Normal astrocytes can undergo anaplasia for unknown reasons and form brain tumors. Astrocytomas are graded from I to IV. As normal brain tissue is displaced by tumor cells, varying signs and symptoms develop, depending on tumor site and size. The goal of surgery, radiation, chemotherapy or a combination of these treatments is to decrease tumor size and improve quality of life and survival time. This article addresses astrocyte cell development, classification of astrocytomas, theories of tumor development, signs and symptoms of astrocytoma, diagnosis and treatment of the astrocytoma. The role of the nurse is explored and two case histories are presented.
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