Journal of neuro-oncology
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Journal of neuro-oncology · Mar 2011
Low-level copy number changes of MYC genes have a prognostic impact in medulloblastoma.
High-level amplifications of MYC genes are associated with poor outcomes in childhood medulloblastoma (MB). However, the occurrence of MYCN and MYCC copy number increases below the intense amplification pattern is rarely reported, and its clinical impact has not yet been determined. Here, we describe this phenomenon and its prognostic significance in a cohort of 29 MB patients. ⋯ We conclude that limited numerical alterations in loci 2p24 (MYCN) and 8q24 (MYCC), as assessed by I-FISH, are present in MB with a higher frequency than high-level amplifications. Poor prognoses were observed in patients with copy number increases in MYC genes. Our data illustrate the importance of further investigations in multicenter trials to better refine the emerging genomic-based prognostic stratification in MB.
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Journal of neuro-oncology · Feb 2011
Case ReportsSpontaneous acute subdural hematoma as the initial manifestation of chronic myeloid leukemia.
Spontaneous acute subdural hematoma is rare and limited to sporadic case reports, associated with neoplasm, aneurysm, arteriovenous malformation and cocaine use. Subdural hematoma has also been reported in association with leukemic malignancies, either during therapy or after diagnosis. However, there are no reports of spontaneous acute subdural hematoma as the primary initial presenting manifestation of a chronic myeloid leukemia. ⋯ Further evaluation revealed that the patient had chronic myeloid leukemia. His peripheral white blood count normalized after Gleevec and hydroxyurea chemotherapy. Furthermore, he had no neurological deficits after his subdural collection was adequately evacuated.
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Journal of neuro-oncology · Feb 2011
Survival among patients with primary central nervous system lymphoma, 1973-2004.
Primary central nervous system lymphoma (PCNSL) is a rare non-Hodgkin's lymphoma that occurs in immunocompetent and human immunodeficiency virus (HIV) patients. Despite treatment advances, previous reports have produced conflicting information about survival trends over time. Using the Surveillance, Epidemiology, and End Results (SEER) data, 2,557 patients diagnosed with PCNSL between 1973 and 2004 were identified and classified by HIV status. ⋯ HIV positivity was a powerful adverse prognostic factor in the overall cohort (HR 4.55 [95% CI 4.01, 5.16]). Despite treatment advances, survival among PCNSL patients in the United States remains poor. However, in the subset of PCNSL patients who are HIV-negative, survival has improved over time.
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Journal of neuro-oncology · Jan 2011
Review Case ReportsTemozolomide for malignant primary spinal cord glioma: an experience of six cases and a literature review.
Malignant primary spinal cord gliomas (PSCGs) are rare, and the optimal treatment for these lesions remains controversial. We report herein treatment outcomes of six malignant PSCGs managed with temozolomide (TMZ)-based multidisciplinary treatment. TMZ was administered concomitantly with fractionated radiotherapy for two newly diagnosed primary spinal cord glioblastoma multiforme (GBM), followed by adjuvant chemotherapy with TMZ. ⋯ TMZ treatment may have a positive effect on control of malignant PSCGs and survival for some patients. Specifically, treatment with TMZ during and after radiation therapy might provide survival benefit to patients with primary spinal cord GBM. A multicenter cooperative investigation for a large-scale study on malignant PSCGs may be required.
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Journal of neuro-oncology · Jan 2011
Post mortem examinations in diffuse intrinsic pontine glioma: challenges and chances.
The concept of organ donation for research purposes has evolved to overcome limited availability of tissue and foster research, in particular in diseases for which surgery is not routinely performed. Diffuse intrinsic pontine glioma (DIPG) is an almost invariably fatal childhood tumour, not amenable to surgery because of its location and infiltrative nature. We report our institutional experience of post mortem brain or tumour donation and its impact on the families. ⋯ None of the families expressed distress from the autopsy, or regretted the choice made. Autopsy limited to the brain did not interfere with the choices related to palliation. The families derived comfort from the hope that scientific breakthroughs could be made and felt that they were helping to make a difference in the future management of DIPG.