Page updated 11/2/2022
What happens once you’ve been diagnosed with GIST
Once you’re been diagnosed, your medical team will take many factors into consideration when deciding what course of treatment will be most effective for your type of GIST. You may have had a biopsy prior to surgery or a biopsy during surgery. The only way to conclusively diagnose GIST is to obtain cells from the abnormal area through a biopsy performed during surgery or by endoscopy (see GIST Diagnosis).
For resectable, primary tumors, surgery is the most common first step. In some cases, neoadjuvant therapy may be prescribed to reduce the size of the tumor prior to surgery. Surgery is the gold standard for treating GIST and offers the best chance of a “cure.” Patients that are cured by surgery tend to be patients with a low risk of recurrence.
Treatment Guidelines & Testing
You can read more about surgery for GIST and read the treatment guidelines developed by the National Comprehensive Cancer Network (NCCN) (U.S.). The treatment guidelines for GIST begin on page 60. The European Society for Medical Oncology (ESMO) for 2021 for GIST are available here.
Identifying and understanding your mutation is an important part of determining an effective treatment plan. Mutational testing is available and typically done at specialty pathology departments. Knowing your mutation will prevent you from living with the side effects of a drug that doesn’t work with your specific mutation. Read more about mutational testing. (Also can be referred to as biomarker testing, genomic profiling, or genomic sequencing.)
What happens after surgery?
Adjuvant therapy may be used to treat patients with a high risk of recurrence or as treatment for metastatic GIST.
Currently, three drugs are commonly used in adjuvant and neoadjuvant therapy to treat GIST.
Gleevec (imatinib) is the ‘first-line’ therapy for the treatment of GIST. It can be used to shrink a tumor prior to surgery, and also after surgery to prevent or delay a recurrence in cases of high-risk GIST.
Sutent (sunitinib) and Stivarga (regorafenib) are the second and third-line therapies, respectively, for patients that are resistant or intolerant to Gleevec.
For patients failing standard treatments, clinical trials are recommended.
On January 9th, 2020, Ayvakit (avapritinib) was approved for treatment in PDGFRA exon 18 mutations.
On May 15th, 2020, Qinlock (ripretinib) was approved for treatment in for 4th line treatment in GIST.
Other treatments include off-label drugs (not always approved by insurance companies), radio-frequency ablation, hepatic arterial embolization, and palliative radiation, which can be effective for the treatment of localized progression
Read more about the importance of mutational testing and managing a recurrence.