Comprehensive Genetic and Histopathologic Study Reveals Three Types of Neuroblastoma Tumors
| Autor: | Andrew D.J. Pearson, Nick Bown, Caroline Ellershaw, Maria Łastowska, James Nicholson, Paul H. Roberts, Michael S. Jackson, Seamus O'Neill, Simon Cotterill, S. Variend, Catherine Cullinane, Katia Mazzocco |
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| Rok vydání: | 2001 |
| Předmět: |
Genetic Markers
Cancer Research medicine.medical_specialty Pathology Adolescent Genes myc Biology medicine.disease_cause Disease-Free Survival Statistics Nonparametric Neuroblastoma Gene duplication medicine Humans Age of Onset Child Survival rate Proportional Hazards Models Mutation Gene Amplification Cytogenetics Infant Prognosis medicine.disease United Kingdom Survival Rate Hyaluronan Receptors Oncology Chromosomes Human Pair 1 Genetic marker Child Preschool Multivariate Analysis Histopathology Age of onset Ireland Chromosomes Human Pair 17 |
| Zdroj: | Journal of Clinical Oncology. 19:3080-3090 |
| ISSN: | 1527-7755 0732-183X |
| DOI: | 10.1200/jco.2001.19.12.3080 |
| Popis: | PURPOSE: To determine the relationship between multiple genetic features, tumor morphology, and prognosis in neuroblastoma. PATIENTS AND METHODS: The genetic alterations and morphologic features that underpin three histopathologic risk classifications were analyzed in 108 neuroblastoma patients. Tumors were subdivided into four groups based on the three most frequent and prognostically significant genetic alterations (17q gain, 1p deletion, and MYCN amplification), and all other genetic, morphologic, and clinical data were analyzed with respect to these groups. RESULTS: Our analyses identify three nonoverlapping tumor types with distinct genetic and morphologic features, defined here as types 1, 2, and 3. Type 1 tumors show none of the three significant genetic alterations and have good prognosis. Both type 2 (17q gain only or 17q gain and 1p del) and type 3 (17q gain, 1p del, and MYCN amplification) tumors progress. However, these tumor types are distinguished clinically by having significantly different median age at diagnosis and median progression-free survival (PFS). Multivariate analysis indicates that 17q gain is the only independent prognostic factor among all genetic, histopathologic, and clinical factors analyzed. Among histopathologic risk systems, the International Neuroblastoma Pathology Classification was the best predictor of PFS. CONCLUSION: Our results indicate that specific combinations of genetic changes in neuroblastoma tumors contribute to distinct morphologic and clinical features. Furthermore, the identification of two genetically and morphologically distinct types of progressing tumors suggests that possibilities for different therapeutic regimens should be investigated. |
| Databáze: | OpenAIRE |
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