Abstract:
OBJECT:Although atypical teratoid/rhabdoid tumor (AT/RT) is known to generate through inactivation of the hSNF5/INI1 gene on chromosome 22q, the downstream molecular mechanism remains unclear. We histologically and molecularly reviewed our pediatric brain tumors for unrecognized AT/RTs and evaluated the role of cyclin D1, a potential molecular target of hSNF5/INI1. METHODS:We analyzed 16 tumors under three years of age: seven medulloblastomas, three anaplastic ependymomas (E IIIs), two each of supratentorial primitive neuroectodermal tumors (sPNETs) and choroid plexus carcinomas (CPCs), and one each of neuroblastoma and pineoblastoma. Immunohistochemistry for glial fibrillary acidic protein, vimentin, epithelial membrane antigen, smooth muscle actin and cyclin D1 was performed. Polymerase chain reaction (PCR)-single-strand conformation polymorphism analysis with direct sequencing, differential PCR and microsatellite analysis were conducted for hSNF5/INI1mutation, homozygous deletion and loss of heterozygosity (LOH) on 22q, respectively. Because of the presence of rhabdoid cells and the polyimmunophenotypic features, the diagnosis was revised to AT/RT in five (31%) tumors, namely, two E IIIs and one each of medulloblastoma, CPC and pineoblastoma. Three of them harbored such hSNF5/INI1 aberrations as germline single base deletion (492/6 delC) and missense mutation (C157T) together with LOH 22q or homozygous deletion. Cyclin D1 was overexpressed in those three tumors but not in the two that lacked hSNF5/INI1 inactivation. CONCLUSION:AT/RT can be misdiagnosed as a variety of tumors, including ependymoma that potentially harbors LOH 22q. Our data indicate that cyclin D1 is a target of hSNF5/INI1in primary tumors.
journal_name
J Neurooncoljournal_title
Journal of neuro-oncologyauthors
Fujisawa H,Misaki K,Takabatake Y,Hasegawa M,Yamashita Jdoi
10.1007/s11060-004-4276-4subject
Has Abstractpub_date
2005-06-01 00:00:00pages
117-24issue
2eissn
0167-594Xissn
1573-7373journal_volume
73pub_type
杂志文章abstract::Intracranial atypical teratoid/rhabdoid tumors (AT/RT) are rare with a poor prognosis. We report one case of a 7-year old girl living over 17 months after the diagnosis of AT/RT in the left frontal lobe. Treatment was partial surgical resection and post-operative radiation therapy. Radiation therapy resulted in comple...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-004-3120-1
更新日期:2005-03-01 00:00:00
abstract::O(6)-methylguanine-DNA-methyltransferase (MGMT) is mainly regulated by cytosine-guanine island promoter methylation that is believed to occur only in neoplastic tissue. The present study was undertaken to investigate whether methylation occurs also in non-neoplastic brains by collecting 45 non-neoplastic brains from a...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-014-1663-3
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:Non-viral manufacturing of CAR T cells via the Sleeping Beauty transposon is cost effective and reduces the risk of insertional mutagenesis from viral transduction. However, the current gold standard methodology requires ex vivo numerical expansion of these cells on artificial antigen-presenting cells (AaPCs...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-019-03311-y
更新日期:2019-12-01 00:00:00
abstract::The new anticancer agent Taxol appears to potentiate the effects of radiation on brain tumor cell lines in vitro and was recently evaluated by our group as a radiosensitizer in a phase I study for primary brain tumors. In that study, we administered Taxol as a three-hour IV infusion repeated every week for six weeks a...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1023/a:1005710710418
更新日期:1998-09-01 00:00:00
abstract::Epigenetic silencing of the O(6)-methylguanine-DNA-methyltransferase (MGMT) gene by promoter methylation is correlated with improved progression-free survival (PFS) and overall survival (OS) in adult patients with newly diagnosed glioblastoma multiforme (GBM) who receive alkylating agents. The aim of this study is to ...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-010-0324-4
更新日期:2011-04-01 00:00:00
abstract::A thirty-year-old male with an intracranial malignant meningioma, first diagnosed 9 years ago, with three recurrences was admitted with a hypoglycemic shock. The blood glucose level was 17 mg/dl, requiring treatment with high doses of intravenous and oral dextrose for improvement. A large metastatic tumor in the liver...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1023/a:1006460827300
更新日期:2000-03-01 00:00:00
abstract::Mitochondrial dysfunction is putatively central to glioblastoma (GBM) pathophysiology but there has been no systematic analysis in GBM of the proteins which are integral to mitochondrial function. Alterations in proteins in mitochondrial enriched fractions from patients with GBM were defined with label-free liquid chr...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-014-1430-5
更新日期:2014-06-01 00:00:00
abstract::Anaplastic mixed gliomas are rare tumors that occur mostly in the cerebral hemispheres. They have a distinctive histological appearance characterized by the presence of two or more glial cellular constituents. The incidence of malignant mixed glioma of the brainstem and posterior fossa is extremely low. The authors re...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1023/a:1005969429013
更新日期:1998-04-01 00:00:00
abstract::Developing and conducting gene therapy clinical trials poses unique challenges which must be addressed to satisfy regulatory requirements and, most importantly, to protect human subjects. Experimental products used for gene transfer studies, such as viral vectors, are often complex and cannot be sterilized or complete...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章,评审
doi:10.1023/b:neon.0000003659.04633.6e
更新日期:2003-12-01 00:00:00
abstract:SUBJECT:To date there is no established tumor marker for the clinical follow-up of glioblastoma, WHO grade IV, (GBM) which constitutes the most frequent and malignant primary brain tumor. However, since there is promising data that the serum glial fibrillary acidic protein (sGFAP) may serve as a biomarker for glial bra...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-018-2898-1
更新日期:2018-09-01 00:00:00
abstract::In the following study, we present our experience in the treatment of PCNSL patients using a multi-step schedule combining chemotherapy and deferred radiotherapy. Patients were treated with two modified M-BACOD cycles and then differently according to radiological response For PR, SD and PD patients, chemotherapy was ...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-006-9276-0
更新日期:2007-05-01 00:00:00
abstract::The utility of current response criteria has not been established in anaplastic astrocytoma (AA). We retrospectively reviewed MR images for 20 patients with AA and compared RANO-based approaches to clinician impression described as follow: (1) standard RANO-based criteria met by growth of or development of new enhanci...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-014-1703-z
更新日期:2015-03-01 00:00:00
abstract::Unexpected early epidural spinal metastasis in a case of osteosarcoma occurred in a patient receiving treatment with cis-diamminedichloroplatinum-II (cisplatin). The initial neurologic symptomatology manifested as paresthesias in the feet which developed 2 months after initiation of treatment (cumulative dose of cispl...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/BF00165378
更新日期:1986-01-01 00:00:00
abstract::Aberrant expression of microRNAs (miRNAs), a class of small non-coding regulatory RNAs, has been implicated in the development and progression of high-grade gliomas. However, the precise mechanistic role of many miRNAs in this disease remains unclear. Here, we investigate the functional role of miR-331-3p in glioblast...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-013-1271-7
更新日期:2014-01-01 00:00:00
abstract::Disparities in American health care based on socially-defined patient characteristics such as race, ethnicity, and socioeconomic position are well-documented. We review differences and disparities in incidence, pathobiology, processes and outcomes of care, and survival based on social factors for brain tumors of all h...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章,评审
doi:10.1007/s11060-009-9840-5
更新日期:2009-05-01 00:00:00
abstract::To assess the clinical outcome and late side effect profile of pencil beam scanning proton therapy (PT) delivered to children with intracranial ependymoma. Between July-2004 and March-2013, 50 patients with intracranial ependymoma (n = 46, grade 3) received involved-field PT at Paul Scherrer Institute (PSI). Median ag...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-016-2090-4
更新日期:2016-05-01 00:00:00
abstract:PURPOSE:To evaluate the effectiveness of complete resection and postoperative radiotherapy in spinal cord ependymomas. METHODS AND MATERIALS:We conducted a retrospective study over 20 patients (13 males and 7 females) with histologically confirmed spinal cord ependymomas between July 1985 and April 2001. Among them, 1...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-004-1386-y
更新日期:2005-01-01 00:00:00
abstract::When SK-N-BE(2) human neuroblastoma cells were exposed for 1h to growth medium supplemented with [14C]arachidonic acid (AA) at final concentrations ranging from 1 microM to 100 microM, an amount of this fatty acid was uptaken ranging form a 2% to a 120% of that present in cells at steady state. As more [14C]AA was upt...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1023/a:1005714320865
更新日期:1997-01-01 00:00:00
abstract::Since its description in 1982, central neurocytoma (CN) has been a relatively innocuous rare tumor of the central nervous system. Comprising of less than 0.5% of all intracranial tumors, most are reported to be slow growing, with low recurrence rates, and a favorable prognosis. Because of its rarity, its cellular biol...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-010-0165-1
更新日期:2010-11-01 00:00:00
abstract::High-grade gliomas release excitotoxic concentrations of glutamate which contributes to their malignant phenotype. To improve our understanding of the mechanisms by which glutamate enhances tumor growth and invasion, we examined alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA)-mediated signaling in glio...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-008-9552-2
更新日期:2008-06-01 00:00:00
abstract::Aneurysmal bone cysts (ABCs) are benign bone lesions with annual incidences ranging from 1.4 to 3.2 cases per million people. Approximately, 10-30% of ABCs are found in the spine. Such lesions are traditionally treated with curettage or other intralesional techniques. Because ABCs can be locally aggressive, intralesio...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章,多中心研究
doi:10.1007/s11060-014-1540-0
更新日期:2014-10-01 00:00:00
abstract::We prospectively evaluated the efficacy and safety of imatinib plus hydroxyurea in patients with progressive/recurrent meningioma. A total of 21 patients with progressive/recurrent meningioma were enrolled in this dual center, single-arm, phase II trial. All patients received 500 mg of hydroxyurea twice a day. Imatini...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章,多中心研究
doi:10.1007/s11060-011-0687-1
更新日期:2012-01-01 00:00:00
abstract:PURPOSE:Most recent literature has confirmed the efficacy of single-session radiosurgery (sRS) in the treatment of intracranial meningioma. Unfortunately, sRS is not always applicable due to large tumor volume and the proximity of the tumor to critical structures. When sRS is not recommended, multi-session radiosurgery...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章,多中心研究
doi:10.1007/s11060-019-03196-x
更新日期:2019-07-01 00:00:00
abstract::Some unresectable and symptomatic meningiomas recur after conventional radiation therapy or stereotactic radiosurgery and are a therapeutic challenge. Evidence-based data from medical therapy for patients with recurrent meningioma can be deemed insufficient. Because of the prevalent expression of PDGF receptors in men...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-012-0896-2
更新日期:2012-09-01 00:00:00
abstract:INTRODUCTION:Intramedullary spinal cord metastases (ISCMs) are rare lesions affecting the spinal cord in patients with disseminated malignancy. Today, due to increased survival, these lesions are more frequently diagnosed. Literature on survival and neurologic outcomes is sparse. Herein, we describe a single institutio...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-019-03105-2
更新日期:2019-04-01 00:00:00
abstract::Although several studies have quantified costs of cancer care; none to date have examined how cancer costs impact family caregivers' emotional health. This study was designed to evaluate how perceptions of economic hardship influence burden, depressive symptoms, and anxiety in family caregivers of persons with a prima...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-008-9778-z
更新日期:2009-07-01 00:00:00
abstract::Intracranial Hodgkin's lymphoma (ICHL) in the HIV positive population is a rarely reported event with only three prior cases reported in the literature. In these prior reports, the outcome of the patients was uncertain or very poor. We report here the case of an HIV seropositive patient with an isolated intracranial r...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-008-9587-4
更新日期:2008-08-01 00:00:00
abstract::Ependymomas are rare neuroepithelial tumors which may arise anywhere along the ventricular system. Tumors arising in the fourth ventricle present unique challenges. Complete tumor resection favors prolonged survival, but may result in inadvertent injury of surrounding neural structures-such as cranial nerve (CN) nucle...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章,评审
doi:10.1007/s11060-016-2198-6
更新日期:2016-11-01 00:00:00
abstract::Gliomatosis cerebri is a rare diffusely infiltrating primary neoplastic glial process of the brain. Our objective is to review clinical presentation, management, and outcome in a large single institution series of gliomatosis cerebri patients. 54 consecutive gliomatosis cerebri cases presenting to Mayo Clinic Rocheste...
journal_title:Journal of neuro-oncology
pub_type: 临床试验,杂志文章
doi:10.1007/s11060-013-1058-x
更新日期:2013-04-01 00:00:00
abstract::Global and gene-specific changes to the epigenome are hallmarks of most tumours including those of pituitary origin, and this fact might offer important clues about diagnostic and therapeutic applications. We performed global DNA methylation screening with 6 invasive and 6 noninvasive nonfunctioning pituitary adenomas...
journal_title:Journal of neuro-oncology
pub_type: 杂志文章
doi:10.1007/s11060-016-2139-4
更新日期:2016-08-01 00:00:00