title: Neurofibromatosis type 1-associated wild-type gastrointestinal stromal tumor treated with anti-IGF-1R monoclonal antibody.
abstract: Gastrointestinal stromal tumors lacking mutations in KIT or PDGFRα are known as wild type (WT) and are less responsive to imatinib. These WT tumors are hypothesized to be dependent on signaling through the insulin-like growth factor 1 receptor (IGF-1R). We report the case of a 29-year-old woman with neurofibromatosis type 1-associated WT GIST treated with an anti-IGF-1R monoclonal antibody. Treatment was ineffective, and the potential basis for lack of response is discussed in the context of IGF-1R expression levels measured within this patients' primary tumor. We suggest that future clinical trials of anti-IGF-1R therapies prospectively determine tumor IGF-1R expression levels for correlation with response to treatment.
authors.affiliation: Department of Medical Oncology, Peter MacCallum Cancer Centre, Locked Bag 1, A'Beckett St, Melbourne, VIC 8006, Australia. fday@med.usyd.edu.au.
authors.forename: F L
authors.initials: FL
authors.lastname: Day
authors.forename: J
authors.initials: J
authors.lastname: Desai
authors.forename: C L
authors.initials: CL
authors.lastname: Corless
authors.forename: M C
authors.initials: MC
authors.lastname: Heinrich
authors.forename: J
authors.initials: J
authors.lastname: Zalcberg
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000276:immunology / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D017526:Receptor, IGF Type 1 / Q000037:antagonists & inhibitors* / Q000276:immunology
chemical_list: D000911:Antibodies, Monoclonal; D017526:Receptor, IGF Type 1
references.citation: Clin Cancer Res. 2008 May 15;14(10):3204-15
references.pmid: 18483389
references.citation: Eur J Cancer. 2007 Sep;43(13):1895-904
references.pmid: 17624760
references.citation: J Clin Oncol. 2003 Dec 1;21(23):4342-9
references.pmid: 14645423
references.citation: Clin Cancer Res. 2008 Jul 15;14(14):4550-5
references.pmid: 18628470
references.citation: Clin Cancer Res. 2010 Apr 15;16(8):2458-65
references.pmid: 20371689
references.citation: Am J Surg Pathol. 2006 Jan;30(1):90-6
references.pmid: 16330947
references.citation: Proc Natl Acad Sci U S A. 2008 Jun 17;105(24):8387-92
references.pmid: 18550829
references.citation: Cancer Res. 2008 Oct 1;68(19):8039-48
references.pmid: 18829562