Fakultäten » Medizinische Fakultät » Kinderspital Zürich: Medizinische Klinik » Onkologie, Abteilung » Prof. Dr. Felix Niggli » Grotzer
| Title / Titel | Neurotrophin-Receptors and Oncogene MYC in Central Nervous System Primitive Neuroectodermal Tumors of Childhood | ||||||
|---|---|---|---|---|---|---|---|
| Abstract (PDF, 14 KB) | |||||||
| Summary / Zusammenfassung | Primitive neuroectodermal brain tumors (PNET) including medulloblastoma (PNET/MB) are the most common malignant childhood brain tumors. Because of the high risk of leptomeningeal dissemination, standard postoperative treatment for PNET includes not only local radiotherapy, but also craniospinal radiotherapy and chemotherapy. Such treatment causes long-term morbidity including endocrine and growth disturbances, as well as neurocognitive dysfunction, which is particularly severe in young children. At the present time, clinical prognostic factors do not identify a "good-outcome" group of PNET patients that can be treated with a substantially less toxic treatment strategy compared to standard treatment. Recently, independent groups found in retrospective studies that high neurotrophin receptor TrkC mRNA expression and low oncogene MYC mRNA expression are powerful independent predictors of a favorable survival outcome in PNET/MB patients. We anticipate that biological prognostic factors, in addition to clinical factors, will define risk groups and help direct therapy decisions for children with PNET. In children whose tumors express favorable biological prognostic markers, a reduction in craniospinal radiation may decrease late treatment complications and increase quality of life without decreasing the chance of survival. To evaluate whether MYC and TrkC can be defined as potential molecular prognostic markers of use for routine disease risk stratification of children with PNET/MB, we will analyze prospectively the expression of MYC and TrkC by quantitative RT-PCR in a multi-institution group effort. | ||||||
| Publications / Publikationen | Grotzer MA, Janss AJ, Fung K-M, Biegel JA, Sutton LN, Rorke LB, Zhao H, Cnaan A, Phillips PC, Lee VMY, Trojanowski JQ. TrkC expression predicts good clinical outcome in primitive neuroectodermal brain tumors. Journal of Clinical Oncology 2000;18:1027-35Grotzer MA, Hogarty MD, Janss AJ, Liu X, Zhao H, Eggert A, Sutton LN, Rorke LB, Brodeur GM, Phillips PC. MYC messenger RNA expression predicts survival outcome in childhood primitive neuroectodermal tumor/medulloblastoma. Clinical Cancer Research 2001;7:2425-33 | ||||||
| Keywords / Suchbegriffe | brain tumor; medulloblastoma; prognosis | ||||||
| Project leadership and contacts / Projektleitung und Kontakte |
|
||||||
| Funding source(s) / Unterstützt durch |
Foundation Krebsliga des Kantons Zürich |
||||||
| Duration of Project / Projektdauer | Jan 2001 to Dec 2003 |