Precision Oncology in Gastrointestinal Stromal Tumors

GIST (gastrointestinal stromal tumors) represent 20% of sarcomatous tumors and 1–2% of primary gastrointestinal cancers. They have an excellent prognosis when localized and resectable, though their prognosis is poor in the metastatic setting, with limited options after the second line until recently...

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Main Authors: Hiba Mechahougui, Montemurro Michael, Alex Friedlaender
Format: Article
Language:English
Published: MDPI AG 2023-04-01
Series:Current Oncology
Subjects:
Online Access:https://www.mdpi.com/1718-7729/30/5/351
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author Hiba Mechahougui
Montemurro Michael
Alex Friedlaender
author_facet Hiba Mechahougui
Montemurro Michael
Alex Friedlaender
author_sort Hiba Mechahougui
collection DOAJ
description GIST (gastrointestinal stromal tumors) represent 20% of sarcomatous tumors and 1–2% of primary gastrointestinal cancers. They have an excellent prognosis when localized and resectable, though their prognosis is poor in the metastatic setting, with limited options after the second line until recently. Four lines are now standard in KIT-mutated GIST and one in PDGFRA-mutated GIST. An exponential growth of new treatments is expected in this era of molecular diagnostic techniques and systematic sequencing. Currently, the main challenge remains the emergence of resistance linked to secondary mutations caused by selective pressure induced by TKIs. Repeating biopsies to tailor treatments might be a step in the right direction, and liquid biopsies at progression may offer a non-invasive alternative. New molecules with wider KIT inhibition are under investigation and could change the catalog and the sequence of existing treatments. Combination therapies may also be an approach to overcome current resistance mechanisms. Here, we review the current epidemiology and biology of GIST and discuss future management options, with an emphasis on genome-oriented therapies.
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spelling doaj.art-55666486f5de473faeb097c0802cc17b2023-11-18T01:01:29ZengMDPI AGCurrent Oncology1198-00521718-77292023-04-013054648466210.3390/curroncol30050351Precision Oncology in Gastrointestinal Stromal TumorsHiba Mechahougui0Montemurro Michael1Alex Friedlaender2Oncology Department, Geneva University Hospital, 1205 Geneva, SwitzerlandGenolier Cancer Center, 1272 Genolier, SwitzerlandOncology Department, Geneva University Hospital, 1205 Geneva, SwitzerlandGIST (gastrointestinal stromal tumors) represent 20% of sarcomatous tumors and 1–2% of primary gastrointestinal cancers. They have an excellent prognosis when localized and resectable, though their prognosis is poor in the metastatic setting, with limited options after the second line until recently. Four lines are now standard in KIT-mutated GIST and one in PDGFRA-mutated GIST. An exponential growth of new treatments is expected in this era of molecular diagnostic techniques and systematic sequencing. Currently, the main challenge remains the emergence of resistance linked to secondary mutations caused by selective pressure induced by TKIs. Repeating biopsies to tailor treatments might be a step in the right direction, and liquid biopsies at progression may offer a non-invasive alternative. New molecules with wider KIT inhibition are under investigation and could change the catalog and the sequence of existing treatments. Combination therapies may also be an approach to overcome current resistance mechanisms. Here, we review the current epidemiology and biology of GIST and discuss future management options, with an emphasis on genome-oriented therapies.https://www.mdpi.com/1718-7729/30/5/351gastrointestinal stromal tumormetastatic GISTprecision oncologytyrosine kinase inhibitorsimatinibKIT
spellingShingle Hiba Mechahougui
Montemurro Michael
Alex Friedlaender
Precision Oncology in Gastrointestinal Stromal Tumors
Current Oncology
gastrointestinal stromal tumor
metastatic GIST
precision oncology
tyrosine kinase inhibitors
imatinib
KIT
title Precision Oncology in Gastrointestinal Stromal Tumors
title_full Precision Oncology in Gastrointestinal Stromal Tumors
title_fullStr Precision Oncology in Gastrointestinal Stromal Tumors
title_full_unstemmed Precision Oncology in Gastrointestinal Stromal Tumors
title_short Precision Oncology in Gastrointestinal Stromal Tumors
title_sort precision oncology in gastrointestinal stromal tumors
topic gastrointestinal stromal tumor
metastatic GIST
precision oncology
tyrosine kinase inhibitors
imatinib
KIT
url https://www.mdpi.com/1718-7729/30/5/351
work_keys_str_mv AT hibamechahougui precisiononcologyingastrointestinalstromaltumors
AT montemurromichael precisiononcologyingastrointestinalstromaltumors
AT alexfriedlaender precisiononcologyingastrointestinalstromaltumors