第671回　千葉県がんセンター研究局集談会のお知らせ

演題： Genome and transcriptome analysis of neuroblastoma

演者： Prof. Gian Paolo Tonini
(Translational Paediatric Oncology,National Cancer Research Institute, Genoa, Italy)

日時： 平成21年3月16日（月曜日） 午後3分～午後4時30分

場所： 千葉県がんセンター研究局　カンファレンスルーム

要旨： Neuroblastic Tumors (NTs) is a group of pediatric cancers with a high incidence in the pre-scholar age. NTs are classified in several histological categories but neuroblastoma, composed by undifferentiated cell without stromal tissue is the most aggressive disseminated tumor occurring in patients over 12 months of age. In 1973 Biedler and coworkers established cell cultures from human metastatic neuroblastoma and opened a new era in the biological and pharmacological study of this cancer. After which several human cell lines were established and transplanted in mice given the opportunity to study the effect of drug both in vitro and in vivo. In meantime patients were carefully managed and risk of relapsed was assessed by age, extension of disease, biological and genomic factors. From several years the Children’s Oncology Group (COG) and the Societé International Oncologie Pediatric European Neuroblastoma (SIOPEN) have reduced therapy of children with localized disease in which surgery is the most effective approach to improve patient survival. Conversely, high-dose therapy and retinoic acid administered after bone marrow ablation are used for patients with advanced disease. COG and SIOPEN groups are working to define new genetic/biological markers in order to more precisely direct therapy in children with neuroblastomas. Information on MYCN copy number, the most important oncogene associated to tumor progression in neuroblastoma, has gained central importance in the decision making process in treatment protocols of patients. Recently, the genome wide analysis by high density oligonucleotide array Comparative Genomic Hybridzation (aCGH) has been also introduced to identify numerical and structural chromosome abnormalities in tumor. A deep analysis of genes associated with neuroblastoma and ganglioneuroblastoma has been performed by laser capture microdissection and microarray technology. Genome and gene expression profiling of NTs allowed to identify candidate neuroblastoma genes associated to tumor progression. Furthermore, the genome wide analysis performed by high density SPNs of familial neuroblastoma have identified the ALK gene as one of the first predisposition gene for neuroblastoma. Finally, the combinatorial genome and transcriptome analysis of NTs show a complex carcinogenesis of this tumors.