GIST: Early Warning Signs and First Steps

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

In the first part of this interview, Gupta discussed the basics of GIST, and now he breaks down his advice for newly diagnosed patients and advises on early warning signs and symptoms patients should watch out for.

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

Gupta: If you're newly diagnosed with GIST and you have access to the pathology report, you can confirm there's a KIT mutation, that's always helpful as the first step, which would be CD117, that's not necessarily going to be positive in all GISTs but in most of them. A patient can look at that themselves these days, everyone has access their own medical records. 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.

What are some early symptoms or signs of GIST?

It can be pretty non-specific, really. We see a lot of patients presenting with abdominal pain, distension, sometimes early satiety, so not feeling as hungry. But any of those symptoms, they can be pretty vague. So if they're prolonged, and they're not going away with supportive care, that's when you want to seek help from your doctor.

Transcript has been edited for clarity and conciseness.

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