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Titel: ETV6-NTRK3 in congenital mesoblastic nephroma: A report of the SIOP/GPOH nephroblastoma study
VerfasserIn: Vokuhl, Christian
Nourkami-Tutdibi, Nasenien
Furtwängler, Rhoikos
Gessler, Manfred
Graf, Norbert
Leuschner, Ivo
Sprache: Englisch
Titel: Pediatric Blood & Cancer
Bandnummer: 65 (2018)
Heft: 4
Verlag/Plattform: Wiley
Erscheinungsjahr: 2017
Freie Schlagwörter: congenital mesoblastic nephroma
GPOH
oncogene
pediatric kidney cancer
SIOP
translocation
DDC-Sachgruppe: 610 Medizin, Gesundheit
Dokumenttyp: Journalartikel / Zeitschriftenartikel
Abstract: Background: Congenital mesoblastic nephroma (MN) is a rare pediatric renal tumor representing approximately 5% of all pediatric renal tumors. Three different types of MN are distinguished histologically: classical, cellular, and mixed. A frequent genetic alteration is the translocation t(12;15) resulting in a fusion of the ETV6 gene on 12p13 and the NTRK3 gene on 15p15 that occurs almost exclusively in cellular MN. The aim of this study was to determine translocation status of a large cohort of MN with respect to tumor subtype and outcome. Procedure: In total, clinical data from 111 patients were available. Sixty-seven tumors were classical MN (51%), 29 cellular MN (31%), and 15 were mixed MN (18%). From these 111 cases, 79 were analyzed by FISH and RT-PCR. Results: All classical and mixed MN were translocation negative. Seventeen out of 29 (58%) cellular MN harbored the ETV6–NTRK3 translocation. Five-year relapse-free survival (RFS) and overall survival (OS) were 93.2% and 96.8% for the complete cohort. All seven relapses occurred in translocation negative tumors. Five-year RFS was significantly inferior for cellular and mixed MN compared to classic MN (89%, 80%, and 98%), whereas 5-year OS was similar (93%, 96%, and 98%). Within the group of cellular MN, patients having translocation-positive tumors had a significantly superior RFS (5-year RFS: 100% vs. 73%). Conclusion: The majority of cellular MNs harbor the ETV6–NTKR3 gene fusion, whereas all classic- and mixed-type MNs were translocation negative. Within the cellular subgroup, patients having translocation-positive tumors had a significantly superior RFS.
DOI der Erstveröffentlichung: 10.1002/pbc.26925
URL der Erstveröffentlichung: https://doi.org/10.1002/pbc.26925
Link zu diesem Datensatz: urn:nbn:de:bsz:291--ds-410343
hdl:20.500.11880/36826
http://dx.doi.org/10.22028/D291-41034
ISSN: 1545-5017
1545-5009
Datum des Eintrags: 10-Nov-2023
Bezeichnung des in Beziehung stehenden Objekts: Supporting Information
In Beziehung stehendes Objekt: https://onlinelibrary.wiley.com/action/downloadSupplement?doi=10.1002%2Fpbc.26925&file=pbc26925-sup-0001-Table-S1.docx
Fakultät: M - Medizinische Fakultät
Fachrichtung: M - Pädiatrie
Professur: M - Prof. Dr. Norbert Graf
M - Prof. Dr. Michael Zemlin
Sammlung:SciDok - Der Wissenschaftsserver der Universität des Saarlandes

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