title: Quadruple wild-type (WT) GIST: defining the subset of GIST that lacks abnormalities of KIT, PDGFRA, SDH, or RAS signaling pathways.
abstract: A subset of GISTs lack mutations in the KIT/PDGFRA or RAS pathways and yet retain an intact succinate dehydrogensase (SDH) complex. We propose that these KIT/PDGFRA/SDH/RAS-P WT GIST tumors be designated as quadruple wild-type (WT) GIST. Further molecular and clinicophatological characterization of quadruple WT GIST will help to determine their prognosis as well as assist in the optimization of medical management, including clinical test of novel therapies.
authors.affiliation: Department of Specialized, Experimental and Diagnostic Medicine, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy; "Giorgio Prodi" Cancer Research Center, University of Bologna, Bologna, Italy.
authors.forename: Maria A
authors.initials: MA
authors.lastname: Pantaleo
authors.forename: Margherita
authors.initials: M
authors.lastname: Nannini
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Corless
authors.forename: Michael C
authors.initials: MC
authors.lastname: Heinrich
mesh_terms: D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis* / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D009154:Mutation; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics* / Q000378:metabolism; D016283:Proto-Oncogene Proteins p21(ras) / Q000235:genetics* / Q000378:metabolism; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics* / Q000378:metabolism; D015398:Signal Transduction; D013385:Succinate Dehydrogenase / Q000235:genetics* / Q000378:metabolism
chemical_list: D013385:Succinate Dehydrogenase; D019009:Proto-Oncogene Proteins c-kit; D020796:Receptor, Platelet-Derived Growth Factor alpha; D016283:Proto-Oncogene Proteins p21(ras)
keywords: BRAF; GIST; NF-1; RAS; SDH deficiency; SDHA; SDHB; gastrointestinal stromal tumors; quadruple WT; quadruple negative; wild type