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Intraventricular hemorrhage caused by peripheral anterior choroidal artery aneurysm rupture: A case report
No full textMGMT promoter methylation in pediatric high-grade gliomas.
No full textWe read with great interest the recent articles of Buttarelli et al. [1] and Srivastava et al. [2] on MGMT status in pediatric high-grade gliomas. The authors observed MGMT promoter methylation in 7/24 (30%) and 10/20 (50%) of the tumors, respectively.
In adults, MGMT promoter methylation is the most promising prognostic marker to predict patients' outcome and it has been observed in the 30–40% of the cases. On the other hand, only sporadic information are available regarding pediatric cases.
In their seminal work, Donson and colleagues [3] observed four of ten (40%) GBM pediatric patients with methylation of the MGMT gene promoter and that the methylated cases were associated with an improved survival time. Moreover, MGMT down-regulation was associated with a good response to temozolomide therapy. Recently, Schlosser and colleagues demonstrated promoter methylation in 77% of pediatric high-grade gliomas [4].
To further investigate the role of MGMT promoter methylation status in this specific group of tumors, we retrospectively surveyed a series of ten pediatric patients surgically treated from January 2001 to September 2008, at the Department of Pediatrics (Pediatric Neurosurgery Unit) of the University Hospital of Padova (seven grade IV and three grade III gliomas). Patients included five males and five females with a mean age of 12.3 years (median 11.5 years) at diagnosis. In five cases, the surgical excision of the tumor mass was partial, in two cases sub-total and in three cases complete. All patients received post-operative chemotherapy with temozolomide (in seven cases combined with other alkylating agents) and radiation therapy. Overall, all patients suffered relapse or progression of the disease: three patients are still alive, whereas seven died. MGMT status assessment was performed as previously described [5], and no case of MGMT promoter methylation has been detected.
This divergent data underlines the fact that we are still far from a well-defined characterization of pediatric cases. Further larger and multi-Institutional studies should investigate and validate the significant role of MGMT promoter methylation status assessment as a potential prognostic factor in pediatric high-grade gliomas, as well as to design different trials and treatment strategies for patients with unmethylated MGMT promoter
Intra-operative 5-aminolevulinic acid (ALA)-induced fluorescence of medulloblastoma: Phenotypic variability and CD133+ expression according to different fluorescence patterns
No full text5-Aminolevulinic acid (5-ALA) fluorescence has been proved advantageous in glioma surgery. Conflicting results have been reported by few studies published in literature about intra-operative 5-ALA-induced fluorescence of medulloblastoma (MDB). The aim of this study is to verify if these conflicting results could be explained by intra-tumoral histological and phenotypic differences. In the present case of a 45-year-old patient affected by a cerebellar MDB, histological analysis of cell phenotype and 5-ALA and CD133 correlation were performed in multiple samples according to different fluorescence patterns. Intra-operatively, the tumor appeared unevenly fluorescent under blue-violet light. Histologically, 5-ALA-intense biopsies from inner areas were characterized by a significant amount of cancer cells, whereas 5-ALA faint regions from peripheral areas displayed normal cerebellar features, with MDB cells infiltrating healthy tissues. Presenting our findings, we show the correlation between different 5-ALA fluorescence patterns of medulloblastoma with specific histological and phenotypical features. Thus, we hypothesize that a distinct relationship between CD133 expression and fluorescence accumulation presented in our study could partially explain the divergent results published in literature
Non-anaplastic pleomorphic xanthoastrocytoma with neuroradiological evidences of leptomeningeal dissemination
No full textCASE REPORT:
A case of a non-anaplastic pleomorphic xanthoastrocytoma (PXA) presenting with leptomeningeal dissemination (LMD) affecting a 9-year-old girl is presented.
DISCUSSION:
The neoplasia in this young girl had the otherwise classical clinical features of PXA: the relatively advanced paediatric age of the patient, the seizures as presenting sign; the primary site in the temporal lobe; and the MRI findings of the partially solid and cystic superficial lesion. Only the tumour involvement of the chiasma and the infundibulus was a relatively unusual finding. In a 5-year period, the tumour underwent malignant transformation, bringing the child to death because of the primary tumour progression. However, the leptomeningeal deposits remained unchanged throughout the clinical course.
CONCLUSION:
To our knowledge, this is the first case of a non-anaplastic PXA presenting with disseminated disease. Thus, it was thought important to describe this case in order to add further information regarding the spectrum of the presenting clinical features of this rare neoplasm and the phenomenon of LMD of non-malignant glioma
Co-localisation of meningioma and craniopharyngioma mimicking a single skull base tumour in an elderly patient.
No full textPOST-SURGICAL HYPERTROPHIC OLIVARY DEGENERATION (HOD): EXPERIENCE IN A PAEDIATRIC POPULATION
No full text
Diffuse Leptomeningeal Glioneuronal Tumors: A New Entity?
No full textThe peculiar radiological and pathological findings of four pediatric cases admitted to the University Hospital of Padua between 1990 and 2007 are described. In all cases, the contrast-enhanced head and spine magnetic resonance images revealed thickened and abnormally enhancing subarachnoid spaces particularly at the level of basal cisterns and interhemispheric fissure. Furthermore, small cystic lesions scattered throughout the brain and mainly in the cerebellum were also visible. All patients were missing a well-defined intraparenchymal mass, although during the follow-up a small intramedullary lesion appeared within the cervical spine in two and subsequently in the frontal horn of the left lateral ventricle in one of those. All patients presented an indolent long-term follow-up. Histologically, the tumors were composed by a monotonous population of cells arranged in straight lines or in small lobules. The cells were characterized by round to oval nuclei with finely granular dispersed chromatin, inconspicuous nucleoli with oligodendrocyte-like features. The morphological and immunohistochemical findings suggested in all cases a "glioneuronal commitment" of the tumors. Because of the unique similar clinical and neuroradiological characteristics, we propose this small series of tumors as a new possible distinct pathological and clinical entity
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