Gupta, a clinician and researcher, is a pediatric oncologist with the Roswell Park Oishei Children’s Cancer and Blood Disorders Program, and he has secondary appointments in the Department of Medicine and the Department of Cancer Genetics and Genomics. He specializes in sarcomas in children, adolescents and young adults (AYA), with a specific interest in Ewing sarcoma, osteosarcoma and soft tissue sarcomas. Additionally sees pediatric and adult patients as well as adults with neurofibromatosis (NF) in Roswell Park’s NF Multidisciplinary Clinic.

Dr. Ajay Gupta 

Dr. Ajay Gupta of the Roswell Park Comprehensive Cancer Center discussed basics of gastrointestinal stromal tumors.

Essential Insights on GIST and Gleevec for Patients

Dr. Ajay Gupta of the Roswell Park Comprehensive Cancer Center in Buffalo, New York, recently sat down for 

 with CURE in order to discuss the basics of gastrointestinal stromal tumors (GIST), highlighting what patients with disease should know and spotlighting crucial treatment advancements.

He discussed the advent of Gleevec (imatinib). As defined by the National Cancer Instittue on its website, Gleevec works by blocking certain proteins made by the BCR::ABL1, PDGFR, or c-KIT oncogene, which may help keep cancer cells from growing and may kill them. It is a type of treatment known as a tyrosine kinase inhibitor, or TKI.

Can you explain what a gastrointestinal stromal tumor (GIST) is and how it is typically treated?

Gastrointestinal stromal tumor is a solid tumor that we usually see in the stomach, but it can occur anywhere else along the gastrointestinal tract. Sometimes we'll see it along the small intestine, but sometimes we'll see something called extragastric intestinal stromal tumors, or AGIST. Overall, they aren’t just found around the stomach, but the stomach is certainly the most common location for them.

The other main thing to know is GISTs are characterized by certain mutations. For example, you have your KIT and EGFR mutations, and these characterize approximately 80% to 90% of GIST tumors. That is something that was highlighted on the cover of Time magazine in 2001 when Gleevec first came out. It specifically has KIT as one of its targets, or what we call a tyrosine kinase inhibitor.

When Gleevec was invented, or when it came out, that was a game-changer for patients with GIST, because they previously had no targeted treatment, and now all of a sudden, you are having patients surviving for long periods of time.

“Breaking Down Gastrointestinal Stromal Tumor Basics,” CURE; https://www.curetoday.com/view/breaking-down-gastrointestinal-stromal-tumor-basics

“Gleevec,” National Cancer Institute; https://www.cancer.gov/publications/dictionaries/cancer-terms/def/gleevec

Transcript has been edited for clarity and conciseness.

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An expert explained what patients newly diagnosed with gastrointestinal stromal tumors need to know.

Expert Advice for Patients With GIST

CURE recently sat down with Dr. Ajay Gupta of the Roswell Park Comprehensive Cancer Center in Buffalo, New York, to discuss what patients should do if they have recently received a diagnosis of gastrointestinal stromal tumors (GIST).

What advice do you have for patients who are newly diagnosed with GIST?

Once you talk to your team and you're confident it's a GIST, the first step is to understand, is it localized, or is it metastatic? So, has it spread? If it's spread, then you're probably talking about lifelong therapy of some kind, Gleevec (imatinib) or not. And in addition, it's one of the tumors where, even if you have metastatic disease, you potentially could get surgical resection of metastatic deposits, depending on how extensive they are, so surgery still has a role to play, even in metastatic disease, where often in other metastatic sarcomas, there isn't a role for surgery. So, asking whether or not the disease is resectable is important, even if you have metastatic disease.

And then, No. 2, I would say, is looking at the mutational profile. So, making sure that your oncologist is sending a mutational profile if the exact mutation isn't known, because that'll help determine whether or not these are targetable, and what kind of drug would be appropriate. There are certain mutations in KIT that are Gleevec-resistant. So you might want to know that. Sometimes we start Gleevec before we have the mutational profile, and then once that comes back, we modify if we need to. Everyone's practice is a little different. So, knowing those two things will be important.

And then, you want to hopefully get an opinion at somewhere where they see a lot of GISTs, that's the other thing I would recommend. Sometimes patients don't realize that instead of going to the next second-line NCCN-approved drug, maybe there's a clinical trial out there looking at the second line and what should be our new standard of care. And so, patients should know that while Gleevec is often our first line option, when you go to second line, often you'll want to look and see if there's a clinical trial.

Dr. Ajay Gupta

Essential Insights on GIST and Gleevec for Patients

Expert Advice for Patients With GIST

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