Pathology of Gastrointestinal Stromal Tumors

Gastrointestinal stromal tumor (GIST) is a well recognized and relatively well understood soft tissue tumor. Early events in GIST development are activating mutations in KIT or PDGFRA , which occur in most GISTs and encode for mutated tyrosine receptor kinases that are therapeutic targets for tyrosi...

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Main Authors: Wai Chin Foo, Bernadette Liegl-Atzwanger, Alexander J. Lazar
Format: Article
Language:English
Published: SAGE Publishing 2012-01-01
Series:Clinical Medicine Insights: Pathology
Online Access:https://doi.org/10.4137/CPath.S9689
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author Wai Chin Foo
Bernadette Liegl-Atzwanger
Alexander J. Lazar
author_facet Wai Chin Foo
Bernadette Liegl-Atzwanger
Alexander J. Lazar
author_sort Wai Chin Foo
collection DOAJ
description Gastrointestinal stromal tumor (GIST) is a well recognized and relatively well understood soft tissue tumor. Early events in GIST development are activating mutations in KIT or PDGFRA , which occur in most GISTs and encode for mutated tyrosine receptor kinases that are therapeutic targets for tyrosine kinase inhibitors, including imatinib and sunitinib. A small minority of GISTs possessing neither KIT nor PDGFRA mutations may have germline mutations in SDH , suggesting a potential role of SDH in the pathogenesis. Immunohistochemical detection of KIT, and more recently DOG1, has proven to be reliable and useful in the diagnosis of GISTs. Because current and future therapies depend on pathologists, it is important that they recognize KIT-negative GISTs, GISTs in specific clinical contexts, GISTs with unusual morphology, and GISTs after treatment. This review focuses on recent developments in the understanding of the biology, immunohistochemical diagnosis, the role of molecular analysis, and risk assessment of GISTs.
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spelling doaj.art-d6539ed8cbdb432ba449ef8f642a0fee2022-12-22T00:39:12ZengSAGE PublishingClinical Medicine Insights: Pathology1179-55572012-01-01510.4137/CPath.S9689Pathology of Gastrointestinal Stromal TumorsWai Chin Foo0Bernadette Liegl-Atzwanger1Alexander J. Lazar2Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA.Institute of Pathology, Medical University of Graz, Graz, Austria.Sarcoma Research Center, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA.Gastrointestinal stromal tumor (GIST) is a well recognized and relatively well understood soft tissue tumor. Early events in GIST development are activating mutations in KIT or PDGFRA , which occur in most GISTs and encode for mutated tyrosine receptor kinases that are therapeutic targets for tyrosine kinase inhibitors, including imatinib and sunitinib. A small minority of GISTs possessing neither KIT nor PDGFRA mutations may have germline mutations in SDH , suggesting a potential role of SDH in the pathogenesis. Immunohistochemical detection of KIT, and more recently DOG1, has proven to be reliable and useful in the diagnosis of GISTs. Because current and future therapies depend on pathologists, it is important that they recognize KIT-negative GISTs, GISTs in specific clinical contexts, GISTs with unusual morphology, and GISTs after treatment. This review focuses on recent developments in the understanding of the biology, immunohistochemical diagnosis, the role of molecular analysis, and risk assessment of GISTs.https://doi.org/10.4137/CPath.S9689
spellingShingle Wai Chin Foo
Bernadette Liegl-Atzwanger
Alexander J. Lazar
Pathology of Gastrointestinal Stromal Tumors
Clinical Medicine Insights: Pathology
title Pathology of Gastrointestinal Stromal Tumors
title_full Pathology of Gastrointestinal Stromal Tumors
title_fullStr Pathology of Gastrointestinal Stromal Tumors
title_full_unstemmed Pathology of Gastrointestinal Stromal Tumors
title_short Pathology of Gastrointestinal Stromal Tumors
title_sort pathology of gastrointestinal stromal tumors
url https://doi.org/10.4137/CPath.S9689
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