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A mechanistic classification of clinical phenotypes in neuroblastoma.
- Source :
-
Science (New York, N.Y.) [Science] 2018 Dec 07; Vol. 362 (6419), pp. 1165-1170. - Publication Year :
- 2018
-
Abstract
- Neuroblastoma is a pediatric tumor of the sympathetic nervous system. Its clinical course ranges from spontaneous tumor regression to fatal progression. To investigate the molecular features of the divergent tumor subtypes, we performed genome sequencing on 416 pretreatment neuroblastomas and assessed telomere maintenance mechanisms in 208 of these tumors. We found that patients whose tumors lacked telomere maintenance mechanisms had an excellent prognosis, whereas the prognosis of patients whose tumors harbored telomere maintenance mechanisms was substantially worse. Survival rates were lowest for neuroblastoma patients whose tumors harbored telomere maintenance mechanisms in combination with RAS and/or p53 pathway mutations. Spontaneous tumor regression occurred both in the presence and absence of these mutations in patients with telomere maintenance-negative tumors. On the basis of these data, we propose a mechanistic classification of neuroblastoma that may benefit the clinical management of patients.<br /> (Copyright © 2018 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works.)
- Subjects :
- Child
Child, Preschool
Disease-Free Survival
Exome genetics
Genome, Human
Humans
Metabolic Networks and Pathways genetics
Mutation
Neuroblastoma drug therapy
Neuroblastoma genetics
Prognosis
Sequence Analysis, DNA
Tumor Suppressor Protein p53 genetics
ras Proteins genetics
Neuroblastoma classification
Neuroblastoma mortality
Telomere Homeostasis genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1095-9203
- Volume :
- 362
- Issue :
- 6419
- Database :
- MEDLINE
- Journal :
- Science (New York, N.Y.)
- Publication Type :
- Academic Journal
- Accession number :
- 30523111
- Full Text :
- https://doi.org/10.1126/science.aat6768