ID

45889

Beschrijving

Principal Investigator: Boris C. Bastian, MD, University of California, San Francisco, CA, USA MeSH: Melanoma https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs000977 Desmoplastic melanoma is an infrequent variant of melanoma with sarcomatous histology, distinct clinical behavior, and unknown pathogenesis. We performed low-coverage genome and high-coverage exome sequencing of 20 desmoplastic melanomas, followed by targeted sequencing of 293 genes to validate candidate genes. A high mutation burden (median 62 mutations/Mb) ranked desmoplastic melanoma among the most highly mutated cancers. Mutation patterns strongly implicate UV-radiation as the dominant mutagen, indicating a superficially located cell of origin. Novel alterations included recurrent promoter mutations of NF-kappa B inhibitor epsilon, NFKBIE (IkBε) in 14.5% of samples. Commonly mutated oncogenes in melanomas, in particular BRAF(V600E) and NRAS(Q61K/R), were absent. Instead, other genetic alterations known to activate the MAPK and PI3K signaling cascades were identified in 73% of samples, affecting NF1, CBL, ERBB2, MAP2K1, MAP3K1, BRAF, EGFR, PTPN11, MET, RAC1, SOS2, NRAS, and PIK3CA, some of which being candidates for targeted therapies. *Reprinted from:* Shain, A. H. et al. Exome sequencing of desmoplastic melanoma identifies recurrent NFKBIE promoter mutations and diverse activating mutations in the MAPK pathway. *Nat. Genet. With permission from Nature Genetics*

Link

dbGaP study = phs000977

Trefwoorden

  1. 25-11-23 25-11-23 - Simon Heim
Houder van rechten

Boris C. Bastian, MD, University of California, San Francisco, CA, USA

Geüploaded op

25 november 2023

DOI

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Licentie

Creative Commons BY 4.0

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dbGaP phs000977 Genetic Analysis of Desmoplastic Melanoma

Eligibility Criteria

Inclusion and exclusion criteria
Beschrijving

Inclusion and exclusion criteria

Alias
UMLS CUI [1,1]
C1512693
UMLS CUI [1,2]
C0680251
Tumors with a diagnosis of desmoplastic melanoma and their matched normals were included in this study. There were no exclusion criteria.
Beschrijving

Tumors with a diagnosis of desmoplastic melanoma and their matched normals were included in this study. There were no exclusion criteria.

Datatype

boolean

Alias
UMLS CUI [1,1]
C0027651
UMLS CUI [1,2]
C1333280
UMLS CUI [1,3]
C0011900
UMLS CUI [1,4]
C0150103
UMLS CUI [1,5]
C1512693

Similar models

Eligibility Criteria

Name
Type
Description | Question | Decode (Coded Value)
Datatype
Alias
Item Group
Inclusion and exclusion criteria
C1512693 (UMLS CUI [1,1])
C0680251 (UMLS CUI [1,2])
Tumors with a diagnosis of desmoplastic melanoma and their matched normals were included in this study. There were no exclusion criteria.
Item
Tumors with a diagnosis of desmoplastic melanoma and their matched normals were included in this study. There were no exclusion criteria.
boolean
C0027651 (UMLS CUI [1,1])
C1333280 (UMLS CUI [1,2])
C0011900 (UMLS CUI [1,3])
C0150103 (UMLS CUI [1,4])
C1512693 (UMLS CUI [1,5])

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