Cases reported "Astrocytoma"

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1/9. 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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keywords = anaplasia
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2/9. Spontaneous anaplasia in pilocytic astrocytoma of cerebellum.

    We report a cystic cerebellar astrocytoma with a mural nodule that contained an additional focus of astrocytoma with the histological features of anaplasia, and showed up to 48% of aneuploid and 3% S-phase cells on flow cytometry. This focus was detectable on the enhanced, as well as non-enhanced T1 and T2 images. This appears to be the first case of pilocytic astrocytoma of cerebellum with focal anaplasia detected on histological and radiological studies.
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keywords = anaplasia
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3/9. 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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keywords = anaplasia
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4/9. Benign brain stem lesions in pediatric patients with neurofibromatosis: case reports.

    The symptoms and clinical courses of 4 patients with neurofibromatosis and lesions of the brain stem identifiable on computed tomographic and/or magnetic resonance imaging scans are described. Two patients underwent biopsy and both had low-grade astrocytomas with no evidence of anaplasia. Both received radiation and chemotherapy. The other 2 patients have been monitored without biopsy or treatment. Three patients are alive and clinically stable, having been followed up for an average of 4 years; neuroimaging studies have shown no change in their tumors. The fourth patient died of a supratentorial primitive neuroectodermal tumor. Imaging studies had shown no change in his brain stem lesion, which at autopsy was found to be a focal collection of fibrillary astrocytes. These data suggest that some patients with brain stem lesions and neurofibromatosis may have a prognosis distinctly different from that of the typical patient with a brain stem glioma. We recommend caution against aggressive operative and adjuvant therapy for brain stem lesions in patients with neurofibromatosis, unless progression of the lesion is documented clinically and/or by imaging.
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keywords = anaplasia
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5/9. Congenital anaplastic astrocytoma with favorable prognosis. Case report.

    A large intracranial tumor that caused macrocrania leading to dystocia was demonstrated by prenatal ultrasound examination. After birth, computerized tomography (CT) confirmed the presence of a giant supratentorial tumor with a large cyst. When the infant was 20 days old, the tumor was radically extirpated. Neuropathological examination revealed an astrocytoma with focal signs of anaplasia showing a macrocyst as well as multiple microcysts resulting from hemorrhages into the tumor. Although no adjuvant radio- or chemotherapy was administered, the child had nearly normal psychomotor development without clinical or CT evidence of tumor recurrence, and is now 3 years old.
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6/9. Extensive advanced maturation of medulloblastoma to astrocytoma and ependymoma.

    Four cases of medulloblastoma in children are reported in which a marked degree of differentiation towards neoplastic adult glioma cells was observed. The original immature and highly malignant tissue had largely disappeared in three and completely disappeared in the fourth. The mature tissues which replaced them included astrocytoma tissues in all four, but ependymoma tissues predominated in two. These cases illustrate a general principle applicable to embryonic tumors, defined as those originating in cells that are still immature. Such maturation and differentiation are presumably associated with a decrease in growth rate and an improved prognosis. A continuation of the process of maturation may account for the unexpectedly favorable course sometimes noted with gliomas in children. The processes of differentiation, dedifferentiation, and anaplasia are discussed, particularly the semantic implications of the terms, and it is suggested that the term "dedifferention" is unfortunate since it may have two different meanings. It may be used as a synonym of anaplasia, or it may imply a progression of change backward on the embryologic pathways along which primitive cells normally mature. The latter may not occur, but the assumption that it does leads to views concerning the glioma group as a whole and the phenomena described in this paper, which we hold to be untentable.
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keywords = anaplasia
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7/9. Malignant transformation of benign gliomas during interstitial irradiation.

    Interstitial curietherapy with 125-iodine is an effective therapeutic option in the treatment of low grade gliomas. Four cases with astrocytoma grade II are presented, where tumour growth characteristics have changed to anaplasia during interstitial irradiation after a primary period of tumour regression. Anaplastic transformation could be due to a radiation effect or an insufficient therapeutic influence of interstitial irradiation on natural tumour progression of glioma growth due to genetic events.
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8/9. Composite pleomorphic xanthoastrocytoma and ganglioglioma: report of four cases and review of the literature.

    Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a rare recently described entity. Only three examples have been documented, one of which showed evidence of malignant transformation. We report an additional four cases and update the literature. With the exception of an 82-year-old man, all patients have been under 30 years of age. The temporal lobe was involved in three cases and cerebellum in another three. Radiologic features were those common to PXA and GG. Histologically, all were "collision tumors" composed of abutting, although spatially distinct, PXA and GG components. In two cases, the second element was only recognized at reexcision or recurrence. Histologic anaplasia, always in the PXA component, was evident as brisk mitotic activity and/or necrosis in five cases. Of the seven patients, one died of disease 17 years after the onset of seizures and after multiple recurrences, the last of which largely resembled glioblastoma. We conclude that the composite PXA-GG is a rare neoplasm that shares many features of its individual components. In addition to its temporal lobe predilection, the cerebellum is frequently affected. As when it occurs in isolation, the PXA component of composite PXA-GG possesses the potential for malignant transformation.
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keywords = anaplasia
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9/9. Gliomatosis cerebri: a brain tumour which is too difficult to treat?

    Gliomatosis cerebri is a rare form of primary diffuse brain tumour first described by Nevin in 1938. It was originally considered to be a post-mortem diagnosis before Troost et al reported a clinically diagnosed case in 1987. However antemortem diagnosis remains difficult due to vague clinical symptoms and often non-specific findings on CT scanning. Gliomatosis cerebri has been classified by the world health organization as an infiltrative tumoural process, which involves at least two, and usually three, lobes of the brain. Magnetic resonance (MR) imaging shows a diffuse infiltrative process with possible mass effect but no necrosis. histology is usually of a low grade astrocytic neoplasm which seemingly infiltrates out of proportion to the degree of anaplasia. We report two patients who presented over the past year, whose clinical and radiological features prompted a preoperative diagnosis of gliomatosis cerebri, confirmed by biopsy.
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