The gene DOG1 is named for its diagnostic relevance to these tumors, which, alongside adrenal paraganglioma and lung chondroma, form Carney’s triad. For 10 points each:
[10h] Name these mesenchymal (“meh-ZANK-uh-mull”) neoplasms believed to arise from interstitial cells of Cajal. Almost 90 percent of all patients with these tumors have mutations in CD117, or c-KIT.
ANSWER: gastrointestinal stromal tumors [or GISTs] (DOG1 stands for “discovered-on-GIST-1.”)
[10m] Because of the similarity between the c-KIT receptor and Abelson’s kinase, GIST patients frequently receive this tyrosine kinase inhibitor after surgery. This drug was developed by Novartis for treating Philadelphia-chromosome-positive leukemia patients.
ANSWER: imatinib [accept Gleevec or Glivec or imatinib mesylate]
[10e] Beyond the c-KIT receptor, imatinib also disrupts activity of a family of growth factors named for these small cells. Those growth factors are synthesized in the alpha granules of these cells alongside proteins like von Willebrand factor.
ANSWER: platelets [accept platelet-derived growth factor or platelet-derived growth factor receptor; prompt on PDGF or PDGFRa]
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