Cases reported "Medulloblastoma"

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1/68. Medullomyoblastoma: A case report.

    Medullomyoblastoma is a rare tumour seen in childhood. We report a medullomyoblastoma occurring in the cerebellar vermis of a 4 year old boy. The light microscopic features, immunohistochemistry and histogenesis are described.
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2/68. Secondary supratentorial anaplastic astrocytoma following treatment of medulloblastoma.

    The development of secondary tumours is a rare but well known late effect of radiation therapy of lesions in the central nervous system. Most radiation-induced tumours are of mesenchymal origin, but on rare occasions gliomas can occur. We describe a patient in whom a supratentorial anaplastic astrocytoma developed 15 years after surgery and radiotherapy for a childhood posterior fossa medulloblastoma. A concise review of the pertinent literature is given.
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3/68. Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population.

    Acquired cerebellar lesions in adults have been shown to produce impairments in higher function as exemplified by the cerebellar cognitive affective syndrome. It is not yet known whether similar findings occur in children with acquired cerebellar lesions, and whether developmental factors influence their presentation. In studies to date, survivors of childhood cerebellar tumours who demonstrate long-term deficits in cognitive functions have undergone surgery as well as cranial irradiation or methotrexate treatment. Investigation of the effects of the cerebellar lesion independent of the known deleterious effects of these agents is important for understanding the role of the cerebellum in cognitive and affective development and for informing treatment and rehabilitation strategies. If the cerebellar contribution to cognition and affect is significant, then damage in childhood may influence a wide range of psychological processes, both as an immediate consequence and as these processes fail to develop normally later on. In this study we evaluated neuropsychological data in 19 children who underwent resection of cerebellar tumours but who received neither cranial irradiation nor methotrexate chemotherapy. Impairments were noted in executive function, including planning and sequencing, and in visual-spatial function, expressive language, verbal memory and modulation of affect. These deficits were common and in some cases could be dissociated from motor deficits. Lesions of the vermis in particular were associated with dysregulation of affect. Behavioural deficits were more apparent in older than younger children. These results reveal that clinically relevant neuropsychological changes may occur following cerebellar tumour resection in children. Age at the time of surgery and the site of the cerebellar lesion influence the neurobehavioural outcome. The results of the present study indicate that the cerebellar cognitive affective syndrome is evident in children as well as in adults, and they provide further clinical evidence that the cerebellum is an essential node in the distributed neural circuitry subserving higher-order behaviours.
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4/68. Cerebellar medulloblastoma in a 65 year old Indian male.

    A case of cerebellar medulloblastoma in a 65 year old male is reported. Cerebellar medulloblastoma is classically seen during childhood, and less than 25% of these tumours are found in adults below 40 years of age. Rarely, cases are reported above the age of 40 years. So far only three cases have been reported in patients aged above 64 years and none of these case reports are from india.
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5/68. MR diffusion imaging and 1H spectroscopy in a child with medulloblastoma. A case report.

    We report on a child with a metastasising medulloblastoma which was assessed by MR diffusion imaging and 1H MR spectroscopy (MRS). Reduced mean apparent diffusion coefficients and a high amount of taurine could be demonstrated. This is the first reported case of high taurine in medulloblastoma in vivo and confirms earlier in vitro findings. It is suggested that the changes on diffusion imaging, possibly reflecting the small-cell histology of the tumour and high taurine in MRS, are indicative of medulloblastoma.
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6/68. medulloblastoma in late adults. Case report and critical review of the literature.

    BACKGROUND: medulloblastoma in late adults (older than 65 years) is an exceptional occurrence; in fact only 8 cases are reported with complete clinical notes in the literature. methods: The authors describe a case of medulloblastoma occurring in a 68-year-old man and analyzed cases reported in the literature. RESULTS: The overall average age of the patients was 72.8 years (range 67-88 years), and the male prevalence of this tumour (70% of cases) seems to be unrelated to age. There is a lateral predominance in late adults (77.7% of cases). Seven patients underwent operation and subsequently these patients underwent a course of radiation therapy. Only two patient was treated with postoperative chemotherapy. Median survival for seven patients treated was 43.2 months (range 23-96 months). CONCLUSIONS: It is interesting to note that: 1) histological analysis revealed a classic type medulloblastoma (88.8% of cases) similar to the children: 2) site of the tumor is lateral similar to the adults (77.7% of cases).
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7/68. Craniospinal radiotherapy for medulloblastoma in a man with severe kyphoscoliosis.

    medulloblastoma is an uncommon tumour in the adult population. Maximum surgical resection followed by craniospinal irradiation with a posterior fossa boost is the standard treatment. We report the case history of an adult with medulloblastoma and severe kyphoscoliosis. The unusual anatomy of the patient posed a technical challenge to the oncologist and the physicist in planning the craniospinal radiotherapy. A shaped spinal field matched to a parallel opposed pair of shaped head fields was used. The technique used in treating this patient was made possible with the use of a multileaf collimation and verification with an electronic portal imaging device. The patient remains well with no sign of relapse 4 years after treatment.
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8/68. Microcephalus, medulloblastoma and excessive toxicity from chemotherapy: an unusual presentation of Fanconi anaemia.

    Fanconi anaemia is a genetically and phenotypically heterogeneous disorder with different forms of clinical presentation. In this case the patient had suffered from microcephalus and delayed motor development from birth, but extensive investigation did not disclose any aetiology. At 3.5 y she developed a cerebellar medulloblastoma which was treated with surgery and chemotherapy. Following chemotherapy with alkylating agents she suffered from severe bone marrow aplasia which caused life-threatening infections, feeding problems and impaired kidney function. Fanconi anaemia was suspected, but it took 2 mo before the chromosome fragility test came out positive. From the moment diagnosis of Fanconi anaemia was made, no further active treatment was given. The patient's condition improved for some time, but she relapsed and died exactly 1 y after the first diagnosis of brain tumour. CONCLUSION: Fanconi anaemia must always be suspected in patients who experience excessive toxicity from chemotherapy regardless of the type of malignancy and congenital malformations.
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9/68. Large cell/anaplastic medulloblastomas and medullomyoblastomas: clinicopathological and genetic features.

    OBJECT: medulloblastoma is the most common malignant central nervous system neoplasm found in children. A distinct variant designated large cell/anaplastic (LC/A) medulloblastoma is characterized by frequent dissemination of cerebrospinal fluid (CSF) at presentation and a more aggressive clinical course. The authors report on their examination of the clinicopathological and genetic features of seven such cases encountered at their institution. methods: Eighty cases of medulloblastomas were reviewed and seven (8.8%) of these were believed to fit the histological and immunohistochemical criteria for LC/A medulloblastoma. In three cases (43%) either desmoplastic or classic medulloblastoma was the underlying subtype, and in two cases (28%) the LC/A tumor was found within the setting of medullomyoblastoma. fluorescence in situ hybridization was used in six of the seven cases to characterize the presence of isochromosome 17q, deletion of chromosome 22q (a deletion characteristically found in atypical teratoid/rhabdoid tumors), and c-myc amplification. The patients' clinical histories revealed CSF dissemination in all cases and lymph node metastasis in one case. Isochromosome 17q was found in five (83%) of six cases. Evidence of chromosomal gains indicated aneuploidy in three tumors (50%), and amplification of c-myc was found in three tumors (50%). No 22q deletions were encountered. CONCLUSIONS: A high percentage of LC/A medulloblastomas arise within a background of typical medulloblastomas or medullomyoblastomas. As is the case in conventional medulloblastomas, the presence of 17q is a common early tumorigenic event; however, in a significant percentage of specimens there is also evidence of aneuploidy and/or amplification of c-myc. These findings indicate that LC/A morphological characteristics reflect a more advanced tumor stage than that found in pure medulloblastomas or in typical medullomyoblastomas.
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keywords = rhabdoid
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10/68. Evolution of malignant cerebellar astrocytoma at the site of a treated medulloblastoma: report of two cases.

    The authors report two unusual cases in which a malignant astrocytoma developed at the site of the excision of an earlier medulloblastoma. While the precise etiology of these astrocytic tumours was unproven, theories of the origins of the second tumours, particularly in relation to the basic nature of medulloblastomas, are discussed.
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ranking = 2
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