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Open AccessJournal ArticleDOI

The genetic landscape of high-risk neuroblastoma

Trevor J. Pugh, +76 more
- 01 Mar 2013 - 
- Vol. 45, Iss: 3, pp 279-284
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TLDR
The authors reported a low median exonic mutation frequency of 0.60 per Mb (0.48 nonsilent) and notably few recurrently mutated genes in high-risk neuroblastoma.
Abstract
Neuroblastoma is a malignancy of the developing sympathetic nervous system that often presents with widespread metastatic disease, resulting in survival rates of less than 50%. To determine the spectrum of somatic mutation in high-risk neuroblastoma, we studied 240 affected individuals (cases) using a combination of whole-exome, genome and transcriptome sequencing as part of the Therapeutically Applicable Research to Generate Effective Treatments (TARGET) initiative. Here we report a low median exonic mutation frequency of 0.60 per Mb (0.48 nonsilent) and notably few recurrently mutated genes in these tumors. Genes with significant somatic mutation frequencies included ALK (9.2% of cases), PTPN11 (2.9%), ATRX (2.5%, and an additional 7.1% had focal deletions), MYCN (1.7%, causing a recurrent p.Pro44Leu alteration) and NRAS (0.83%). Rare, potentially pathogenic germline variants were significantly enriched in ALK, CHEK2, PINK1 and BARD1. The relative paucity of recurrent somatic mutations in neuroblastoma challenges current therapeutic strategies that rely on frequently altered oncogenic drivers.

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Journal ArticleDOI

Signatures of mutational processes in human cancer

Ludmil B. Alexandrov, +84 more
- 22 Aug 2013 - 
TL;DR: It is shown that hypermutation localized to small genomic regions, ‘kataegis’, is found in many cancer types, and this results reveal the diversity of mutational processes underlying the development of cancer.

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Mutational heterogeneity in cancer and the search for new cancer genes

TL;DR: The MutSigCV method as mentioned in this paper applies mutational heterogeneity to exome sequences from 3,083 tumour-normal pairs and discovers extraordinary variation in mutation frequency and spectrum within cancer types, which sheds light on mutational processes and disease aetiology.
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Landscape of Microsatellite Instability Across 39 Cancer Types.

TL;DR: Evidence of as-yet-unappreciated MSI in several types of cancer support an expanded role for clinical MSI testing across multiple cancer types as patients with MSI-positive tumors are predicted to benefit from novel immunotherapies in clinical trials.
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Neuroblastoma: developmental biology, cancer genomics and immunotherapy

TL;DR: The key discoveries in the developmental biology, molecular genetics and immunology of neuroblastoma are discussed, as well as new translational tools for bringing these promising scientific advances into the clinic.
References
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Journal ArticleDOI

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TL;DR: SAMtools as discussed by the authors implements various utilities for post-processing alignments in the SAM format, such as indexing, variant caller and alignment viewer, and thus provides universal tools for processing read alignments.
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