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Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs

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Resumo:Aims: Gastrointestinal stromal tumours (GISTs) are commonly driven by oncogenic mutations in KIT and PDGFRA. However, 10-40% of these patients are wild-type for these genes. The prognostic significance of wild-type GISTs is controversial, and they rarely respond to imatinib. The aim of this study was to elucidate the molecular lesions underlying wild-type GISTs tumorigenesis. Methods and results: Twenty-nine KIT and PDGFRA wild-type GISTs were re-assessed for the presence of 'cryptic'KIT exon 11 duplications. Using a specific polymerase chain reaction assay, three previously undetected mutations were identified. In the remaining 26 wild-type GISTs, KIT, stem cell factor (SCF), phospho-KIT and phospho-ERK expression was evaluated by immunohistochemistry. Samples were screened for gain-of-function mutations in the mitogen-activated protein kinase (MAPK) cascade. KIT and SCF co-expression associated with KIT activation was observed in approximately 30% of cases. Furthermore, phospho-ERK expression showed that MAPK is activated in approximately 30% of cases. None of RAS family (H-, K- and N-RAS) oncogenes exhibited activating mutations, whereas BRAF mutations were found in approximately 4% of cases. Conclusions: In the absence of RAS mutations, MAPK could be activated through SCF/KIT autocrine/paracrine mechanisms and/or mutated BRAF in a subset of KIT/PDGFRA wild-type GISTs.
Autores principais:Martinho, Olga
Outros Autores:Gouveia, António; Pereira, Marta Sofia Carvalho Ribeiro Viana; Silva, Paula; Pimenta, Amadeu; Reis, R. M.; Lopes, José Manuel
Assunto:Adult Aged Aged, 80 and over Biomarkers, Tumor Extracellular Signal-Regulated MAP Kinases Female Gastrointestinal Neoplasms Gastrointestinal Stromal Tumors Humans Male Middle Aged Mitogen-Activated Protein Kinase Kinases Mutations Prognosis Proto-Oncogene Proteins B-raf Proto-Oncogene Proteins c-kit Proto-Oncogene Proteins p21(ras) Receptor, Platelet-Derived Growth Factor alpha Retrospective Studies Signal Transduction Activation GISTs MAP kinase Wild-type
Ano:2009
País:Portugal
Tipo de documento:artigo
Tipo de acesso:acesso restrito
Instituição associada:Universidade do Minho
Idioma:inglês
Origem:RepositóriUM - Universidade do Minho
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author Martinho, Olga
author2 Gouveia, António
Pereira, Marta Sofia Carvalho Ribeiro Viana
Silva, Paula
Pimenta, Amadeu
Reis, R. M.
Lopes, José Manuel
author2_role author
author
author
author
author
author
author_facet Martinho, Olga
Gouveia, António
Pereira, Marta Sofia Carvalho Ribeiro Viana
Silva, Paula
Pimenta, Amadeu
Reis, R. M.
Lopes, José Manuel
author_role author
contributor_name_str_mv RepositóriUM - Universidade do Minho
country_str PT
creators_json_txt [{\"Person.name\":\"Martinho, Olga\"},{\"Person.name\":\"Gouveia, António\"},{\"Person.name\":\"Pereira, Marta Sofia Carvalho Ribeiro Viana\"},{\"Person.name\":\"Silva, Paula\"},{\"Person.name\":\"Pimenta, Amadeu\"},{\"Person.name\":\"Reis, R. M.\"},{\"Person.name\":\"Lopes, José Manuel\"}]
datacite.contributors.contributor.contributorName.fl_str_mv RepositóriUM - Universidade do Minho
datacite.creators.creator.creatorName.fl_str_mv Martinho, Olga
Gouveia, António
Pereira, Marta Sofia Carvalho Ribeiro Viana
Silva, Paula
Pimenta, Amadeu
Reis, R. M.
Lopes, José Manuel
datacite.date.Accepted.fl_str_mv 2009-07-01T00:00:00Z
datacite.date.embargoed.fl_str_mv 10000-01-01T00:00:00Z
datacite.rights.fl_str_mv http://purl.org/coar/access_right/c_16ec
datacite.subjects.subject.fl_str_mv Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Extracellular Signal-Regulated MAP Kinases
Female
Gastrointestinal Neoplasms
Gastrointestinal Stromal Tumors
Humans
Male
Middle Aged
Mitogen-Activated Protein Kinase Kinases
Mutations
Prognosis
Proto-Oncogene Proteins B-raf
Proto-Oncogene Proteins c-kit
Proto-Oncogene Proteins p21(ras)
Receptor, Platelet-Derived Growth Factor alpha
Retrospective Studies
Signal Transduction
Activation
GISTs
MAP kinase
Wild-type
datacite.titles.title.fl_str_mv Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
dc.contributor.none.fl_str_mv RepositóriUM - Universidade do Minho
dc.creator.none.fl_str_mv Martinho, Olga
Gouveia, António
Pereira, Marta Sofia Carvalho Ribeiro Viana
Silva, Paula
Pimenta, Amadeu
Reis, R. M.
Lopes, José Manuel
dc.date.Accepted.fl_str_mv 2009-07-01T00:00:00Z
dc.date.embargoed.fl_str_mv 10000-01-01T00:00:00Z
dc.format.none.fl_str_mv application/pdf
dc.identifier.none.fl_str_mv https://hdl.handle.net/1822/67600
dc.language.none.fl_str_mv eng
dc.publisher.none.fl_str_mv Blackwell Publishing
dc.rights.none.fl_str_mv http://purl.org/coar/access_right/c_16ec
dc.subject.none.fl_str_mv Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Extracellular Signal-Regulated MAP Kinases
Female
Gastrointestinal Neoplasms
Gastrointestinal Stromal Tumors
Humans
Male
Middle Aged
Mitogen-Activated Protein Kinase Kinases
Mutations
Prognosis
Proto-Oncogene Proteins B-raf
Proto-Oncogene Proteins c-kit
Proto-Oncogene Proteins p21(ras)
Receptor, Platelet-Derived Growth Factor alpha
Retrospective Studies
Signal Transduction
Activation
GISTs
MAP kinase
Wild-type
dc.title.fl_str_mv Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
dc.type.none.fl_str_mv http://purl.org/coar/resource_type/c_6501
description Aims: Gastrointestinal stromal tumours (GISTs) are commonly driven by oncogenic mutations in KIT and PDGFRA. However, 10-40% of these patients are wild-type for these genes. The prognostic significance of wild-type GISTs is controversial, and they rarely respond to imatinib. The aim of this study was to elucidate the molecular lesions underlying wild-type GISTs tumorigenesis. Methods and results: Twenty-nine KIT and PDGFRA wild-type GISTs were re-assessed for the presence of 'cryptic'KIT exon 11 duplications. Using a specific polymerase chain reaction assay, three previously undetected mutations were identified. In the remaining 26 wild-type GISTs, KIT, stem cell factor (SCF), phospho-KIT and phospho-ERK expression was evaluated by immunohistochemistry. Samples were screened for gain-of-function mutations in the mitogen-activated protein kinase (MAPK) cascade. KIT and SCF co-expression associated with KIT activation was observed in approximately 30% of cases. Furthermore, phospho-ERK expression showed that MAPK is activated in approximately 30% of cases. None of RAS family (H-, K- and N-RAS) oncogenes exhibited activating mutations, whereas BRAF mutations were found in approximately 4% of cases. Conclusions: In the absence of RAS mutations, MAPK could be activated through SCF/KIT autocrine/paracrine mechanisms and/or mutated BRAF in a subset of KIT/PDGFRA wild-type GISTs.
dirty 0
eu_rights_str_mv restrictedAccess
format article
fulltext.url.fl_str_mv https://repositorium.uminho.pt/bitstreams/9a72f975-6eee-4013-88e9-b805f51b31c8/download
id rum_a49eb03a76a45878e833ea09fe03abfc
identifier.url.fl_str_mv https://hdl.handle.net/1822/67600
instacron_str repositorium
institution Universidade do Minho
instname_str Universidade do Minho
language eng
network_acronym_str rum
network_name_str RepositóriUM - Universidade do Minho
oai_identifier_str oai:repositorium.uminho.pt:1822/67600
organization_str_mv urn:organizationAcronym:repositorium
person_str_mv Martinho, Olga
Gouveia, António
Pereira, Marta Sofia Carvalho Ribeiro Viana
Silva, Paula
Pimenta, Amadeu
Reis, R. M.
Lopes, José Manuel
publishDate 2009
publisher.none.fl_str_mv Blackwell Publishing
reponame_str RepositóriUM - Universidade do Minho
repository_id_str urn:repositoryAcronym:rum
service_str_mv urn:repositoryAcronym:rum
spelling engBlackwell PublishingporAims: Gastrointestinal stromal tumours (GISTs) are commonly driven by oncogenic mutations in KIT and PDGFRA. However, 10-40% of these patients are wild-type for these genes. The prognostic significance of wild-type GISTs is controversial, and they rarely respond to imatinib. The aim of this study was to elucidate the molecular lesions underlying wild-type GISTs tumorigenesis. Methods and results: Twenty-nine KIT and PDGFRA wild-type GISTs were re-assessed for the presence of 'cryptic'KIT exon 11 duplications. Using a specific polymerase chain reaction assay, three previously undetected mutations were identified. In the remaining 26 wild-type GISTs, KIT, stem cell factor (SCF), phospho-KIT and phospho-ERK expression was evaluated by immunohistochemistry. Samples were screened for gain-of-function mutations in the mitogen-activated protein kinase (MAPK) cascade. KIT and SCF co-expression associated with KIT activation was observed in approximately 30% of cases. Furthermore, phospho-ERK expression showed that MAPK is activated in approximately 30% of cases. None of RAS family (H-, K- and N-RAS) oncogenes exhibited activating mutations, whereas BRAF mutations were found in approximately 4% of cases. Conclusions: In the absence of RAS mutations, MAPK could be activated through SCF/KIT autocrine/paracrine mechanisms and/or mutated BRAF in a subset of KIT/PDGFRA wild-type GISTs.application/pdfporLow frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTsMartinho, OlgaGouveia, AntónioPereira, Marta Sofia Carvalho Ribeiro VianaSilva, PaulaPimenta, AmadeuReis, R. M.Lopes, José ManuelHostingInstitutionOrganizationalRepositóriUM - Universidade do Minhoe-mailmailto:repositorium@usdb.uminho.ptrepositorium@usdb.uminho.ptPMID19614767ISSNIsPartOf0309-0167EISSNIsPartOf1365-2559DOIIsPartOf10.1111/j.1365-2559.2009.03323.x2009-072009-07-01T00:00:00Z10000-01-01T00:00:00ZHandlehttps://hdl.handle.net/1822/67600http://purl.org/coar/access_right/c_16ecrestricted accessAdultAgedAged, 80 and overBiomarkers, TumorExtracellular Signal-Regulated MAP KinasesFemaleGastrointestinal NeoplasmsGastrointestinal Stromal TumorsHumansMaleMiddle AgedMitogen-Activated Protein Kinase KinasesMutationsPrognosisProto-Oncogene Proteins B-rafProto-Oncogene Proteins c-kitProto-Oncogene Proteins p21(ras)Receptor, Platelet-Derived Growth Factor alphaRetrospective StudiesSignal TransductionActivationGISTsMAP kinaseWild-type706618 bytesliteraturehttp://purl.org/coar/resource_type/c_6501journal articlehttp://purl.org/coar/access_right/c_f1cfapplication/pdffulltexthttps://repositorium.uminho.pt/bitstreams/9a72f975-6eee-4013-88e9-b805f51b31c8/download
spellingShingle Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
Martinho, Olga
Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Extracellular Signal-Regulated MAP Kinases
Female
Gastrointestinal Neoplasms
Gastrointestinal Stromal Tumors
Humans
Male
Middle Aged
Mitogen-Activated Protein Kinase Kinases
Mutations
Prognosis
Proto-Oncogene Proteins B-raf
Proto-Oncogene Proteins c-kit
Proto-Oncogene Proteins p21(ras)
Receptor, Platelet-Derived Growth Factor alpha
Retrospective Studies
Signal Transduction
Activation
GISTs
MAP kinase
Wild-type
status SINGLETON
subject.fl_str_mv Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Extracellular Signal-Regulated MAP Kinases
Female
Gastrointestinal Neoplasms
Gastrointestinal Stromal Tumors
Humans
Male
Middle Aged
Mitogen-Activated Protein Kinase Kinases
Mutations
Prognosis
Proto-Oncogene Proteins B-raf
Proto-Oncogene Proteins c-kit
Proto-Oncogene Proteins p21(ras)
Receptor, Platelet-Derived Growth Factor alpha
Retrospective Studies
Signal Transduction
Activation
GISTs
MAP kinase
Wild-type
title Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
title_full Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
title_fullStr Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
title_full_unstemmed Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
title_short Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
title_sort Low frequency of MAP kinase pathway alterations in KIT and PDGFRA wild-type GISTs
topic Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Extracellular Signal-Regulated MAP Kinases
Female
Gastrointestinal Neoplasms
Gastrointestinal Stromal Tumors
Humans
Male
Middle Aged
Mitogen-Activated Protein Kinase Kinases
Mutations
Prognosis
Proto-Oncogene Proteins B-raf
Proto-Oncogene Proteins c-kit
Proto-Oncogene Proteins p21(ras)
Receptor, Platelet-Derived Growth Factor alpha
Retrospective Studies
Signal Transduction
Activation
GISTs
MAP kinase
Wild-type
topic_facet Adult
Aged
Aged, 80 and over
Biomarkers, Tumor
Extracellular Signal-Regulated MAP Kinases
Female
Gastrointestinal Neoplasms
Gastrointestinal Stromal Tumors
Humans
Male
Middle Aged
Mitogen-Activated Protein Kinase Kinases
Mutations
Prognosis
Proto-Oncogene Proteins B-raf
Proto-Oncogene Proteins c-kit
Proto-Oncogene Proteins p21(ras)
Receptor, Platelet-Derived Growth Factor alpha
Retrospective Studies
Signal Transduction
Activation
GISTs
MAP kinase
Wild-type
url https://hdl.handle.net/1822/67600
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