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Histologic and Molecular Characterization of Solid Pediatric Tumors

小儿实体瘤的组织学和分子特征

基本信息

批准号：
8554160
负责人：
MARIA TSOKOS
金额：
$ 24.09万
依托单位：
DIVISION OF CLINICAL SCIENCES - NCI
依托单位国家：
美国
项目类别：
财政年份：
资助国家：
美国
起止时间：
至
项目状态：
未结题

来源：
https://reporter.nih.gov/project-details/8554160
关键词：
Area Benign Childhood Childhood Solid Neoplasm Childhood Synovial Sarcoma Classification Clinical Protocols Clinical Trials Data Diagnosis EGFR Gene Amplification Educational process of instructing Epidermal Growth Factor Receptor Evaluation Ewings sarcoma Ganglioside GD2 Gastrointestinal Stromal Tumors Gene Mutation Genes Glioma Histologic Hypoxia Lead Malignant - descriptor Manuscripts Molecular Mutation Nerve Sheath Tumors Neurofibromatosis 1 Pathogenesis Pathology Patients Pediatric Neoplasm Play Pontine structure Positron-Emission Tomography Preparation Proteins Proto-Oncogene Protein c-kit Role Solid Neoplasm Specimen Staging Staining method Stains Succinate Dehydrogenase Tissues Tumor Pathology Up-Regulation age group design malignant phenotype molecular marker nestin protein novel osteosarcoma outcome forecast sarcoma therapeutic target translational study tumor tumor progression uptake

项目摘要

The following are examples of recent translational studies which we have either initiated or played a major collaborative role and which have been completed and are at the stage of preparation of a manuscript : - We studied nestin expression in benign and malignant nerve sheath tumors from patients with neurofibromatosis 1 and found its correlation with a malignant phenotype. We also found increased expression of nestin in histologically atypical areas of otherwise benign tumors that correlate with increased PET uptake, suggesting that it may serve as a marker of tumor progression. - We evaluated expression of the ganglioside GD2 in large number of solid pediatric tumors and found high expression in osteosarcoma and Ewing sarcoma. Our data will help design a clinical trial targeting GD2 in these tumors. - We evaluated NY-ESO expression pediatric synovial sarcomas and other solid tumors in this age group, which will lead to a new clinical trial at the NCI. - We found that c-kit negative gastrointestinal stromal tumors (GIST) with mutation in the succinate dehydrogenase (SDH)B gene, which lack expression of SDHB, show high expression of EGFR, but lack EGFR gene amplification or mutation. These data suggest that c-kit negative (pediatric) GIST may be yet another tumor example of translational upregulation of EGFR by tumor hypoxia.- We collaborate on a study that seeks to identify molecular therapeutic targets in pediatric pontine gliomas, by performing immunohistochemical staining and evaluation of a panel of proteins

以下是最近的翻译研究的例子，我们已经开始或发挥了重要的合作作用，并已完成，并在准备阶段的手稿：-我们研究了巢蛋白表达的良性和恶性神经鞘瘤患者神经纤维瘤1，并发现其与恶性表型的相关性。我们还发现巢蛋白在其他良性肿瘤的组织学非典型区域的表达增加，这与PET摄取增加相关，表明它可能作为肿瘤进展的标志物。- 我们评估了神经节苷脂GD 2在大量儿童实体肿瘤中的表达，发现其在骨肉瘤和尤文肉瘤中高表达。我们的数据将有助于设计针对这些肿瘤中GD 2的临床试验。- 我们评估了NY-ESO在这个年龄组的儿童滑膜肉瘤和其他实体瘤中的表达，这将导致在NCI进行新的临床试验。- 我们发现，c-kit阴性的胃肠道间质瘤（GIST）与突变的琥珀酸脱氢酶（SDH）B基因，缺乏表达的SDHB，表现出高表达的EGFR，但缺乏EGFR基因扩增或突变。这些数据表明，c-kit阴性（儿科）GIST可能是肿瘤缺氧导致EGFR翻译上调的另一个肿瘤实例。我们合作的一项研究，旨在确定分子治疗的目标，在儿童脑桥胶质瘤，通过进行免疫组化染色和评估的一组蛋白质