0 Ratings

ID

45807

Description

Principal Investigator: Adam Resnick, PhD, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, PA, USA MeSH: Glioma,Astrocytoma https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs001054 Pediatric low-grade gliomas (PLGGs) are the most common pediatric brain-tumor, with more than ten histologic subtypes recognized by the World Health Organization. We performed a genomic analysis of 230 PLGGs of which 73 had whole genome/RNA sequencing performed and show that MYB-QKI fusions define the seizure associated tumor, Angiocentric Glioma (AG). MYB-QKI fusions present in AGs contribute to tumorigenesis through three mechanisms: MYB activation by truncation, enhancer translocation driving aberrant MYB-QKI expression, and hemizygous loss of QKI, a tumor suppressor gene. Such interplay between three oncogenic mechanisms has diagnostic and therapeutic implications in AGs, and illustrates the functional complexity associated with rearrangements in cancer.

Link

dbGaP-study=phs001054

Keywords

  1. 6/23/23 6/23/23 - Chiara Middel
Copyright Holder

Adam Resnick, PhD, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, PA, USA

Uploaded on

June 23, 2023

DOI

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License

Creative Commons BY 4.0

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    dbGaP phs001054 Genomic Characterization of Pediatric Low-Grade Gliomas

    Sample ID, analyte type, tumor status of sample, histological type, whether the sample is recurrent tumor, and body site where sample was collected of participants with pediatric low-grade gliomas and involved in the "Genomic Characterization of Pediatric Low-Grade Gliomas" project.

    pht005279
    Description

    pht005279

    Alias
    UMLS CUI [1,1]
    C3846158
    De-identified sample ID
    Description

    SAMPID

    Data type

    string

    Alias
    UMLS CUI [1,1]
    C4684638
    UMLS CUI [1,2]
    C1299222
    Sample content analyzed
    Description

    ANALYTE_TYPE

    Data type

    string

    Alias
    UMLS CUI [1,1]
    C2347026
    UMLS CUI [1,2]
    C0456205
    UMLS CUI [1,3]
    C0936012
    Tumor status
    Description

    IS_TUMOR

    Data type

    text

    Alias
    UMLS CUI [1,1]
    C0475752
    Cell or tissue type or subtype of sample
    Description

    HISTOLOGICAL_TYPE

    Data type

    string

    Alias
    UMLS CUI [1,1]
    C2347026
    UMLS CUI [1,2]
    C0007634
    UMLS CUI [1,3]
    C0332307
    UMLS CUI [2,1]
    C2347026
    UMLS CUI [2,2]
    C0007634
    UMLS CUI [2,3]
    C0449560
    UMLS CUI [3,1]
    C1292533
    UMLS CUI [3,2]
    C0332307
    UMLS CUI [4,1]
    C1292533
    UMLS CUI [4,2]
    C0449560
    Whether the sample is from a recurrent tumor
    Description

    IS_RECURRENT

    Data type

    text

    Alias
    UMLS CUI [1,1]
    C2347026
    UMLS CUI [1,2]
    C0277556
    UMLS CUI [1,3]
    C1512698
    Body site where sample was collected
    Description

    BODY_SITE

    Data type

    string

    Alias
    UMLS CUI [1,1]
    C0449705

    Similar models

    Sample ID, analyte type, tumor status of sample, histological type, whether the sample is recurrent tumor, and body site where sample was collected of participants with pediatric low-grade gliomas and involved in the "Genomic Characterization of Pediatric Low-Grade Gliomas" project.

    Name
    Type
    Description | Question | Decode (Coded Value)
    Data type
    Alias
    Item Group
    pht005279
    C3846158 (UMLS CUI [1,1])
    SAMPID
    Item
    De-identified sample ID
    string
    C4684638 (UMLS CUI [1,1])
    C1299222 (UMLS CUI [1,2])
    ANALYTE_TYPE
    Item
    Sample content analyzed
    string
    C2347026 (UMLS CUI [1,1])
    C0456205 (UMLS CUI [1,2])
    C0936012 (UMLS CUI [1,3])
    Item
    Tumor status
    text
    C0475752 (UMLS CUI [1,1])
    Code List
    Tumor status
    CL Item
    Is not a tumor (N)
    C0027651 (UMLS CUI [1,1])
    C1518422 (UMLS CUI [1,2])
    CL Item
    Is tumor (Y)
    C0027651 (UMLS CUI [1,1])
    HISTOLOGICAL_TYPE
    Item
    Cell or tissue type or subtype of sample
    string
    C2347026 (UMLS CUI [1,1])
    C0007634 (UMLS CUI [1,2])
    C0332307 (UMLS CUI [1,3])
    C2347026 (UMLS CUI [2,1])
    C0007634 (UMLS CUI [2,2])
    C0449560 (UMLS CUI [2,3])
    C1292533 (UMLS CUI [3,1])
    C0332307 (UMLS CUI [3,2])
    C1292533 (UMLS CUI [4,1])
    C0449560 (UMLS CUI [4,2])
    Item
    Whether the sample is from a recurrent tumor
    text
    C2347026 (UMLS CUI [1,1])
    C0277556 (UMLS CUI [1,2])
    C1512698 (UMLS CUI [1,3])
    Code List
    Whether the sample is from a recurrent tumor
    CL Item
    Is a primarily resected tumor (N)
    CL Item
    not applicable (NA)
    C1272460 (UMLS CUI [1,1])
    CL Item
    Is a recurrent tumor (Y)
    BODY_SITE
    Item
    Body site where sample was collected
    string
    C0449705 (UMLS CUI [1,1])

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