Succinate dehydrogenase-deficient GISTs are characterized by IGF1R overexpression

Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc
Angela ChouAnthony J Gill

Abstract

Succinate dehydrogenase-deficient gastrointestinal stromal tumors (GISTs) demonstrate unique pathological and clinical features, including the absence of activating mutations of KIT and PDGFRA, and primary resistance to imatinib. They arise exclusively in the stomach and account for 5-7.5% of all adult stomach GISTs and the great majority of these tumors in childhood. Insulin-like growth factor 1 receptor (IGF1R) overexpression has been associated with wild-type and pediatric GISTs. We propose that IGF1R overexpression is a feature of succinate dehydrogenase-deficient GISTs as a group. We assessed succinate dehydrogenase complex subunit B (SDHB) and IGF1R expression by immunohistochemistry in eight known succinate dehydrogenase-deficient GISTs, three GISTs arising in the setting of neurofibromatosis type 1 syndrome and 40 unselected GISTs. Selected KIT and PDGFRA exons were amplified and sequenced from formalin-fixed paraffin-embedded tumor samples. All eight succinate dehydrogenase-deficient tumors were wild-type for KIT and PDGFRA, succinate dehydrogenase B negative and demonstrated IGF1R overexpression. The three neurofibromatosis-related tumors were succinate dehydrogenase B positive and IGF1R negative. Of the 40 unselected...Continue Reading

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Citations

Apr 25, 2013·European Journal of Human Genetics : EJHG·Maria A PantaleoUNKNOWN GIST Study Group
Jul 9, 2013·Journal of Medical Genetics·Margherita NanniniMaria A Pantaleo
Sep 23, 2014·BMC Cancer·Margherita NanniniMaria A Pantaleo
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