ABOUT GIST
—A rare cancer that affects your GI tract

 

are tumors that show up in the , often in the stomach or small intestine. About 4,000-6,000 adults in the US are diagnosed with GIST every year.

The cause of GIST is unknown. There are no known lifestyle factors that cause GIST (such as diet or smoking).

 

GIST: Get the Basics

Arun Singh, MD: The title of today’s webinar is Understanding Your GIST Journey and how QINLOCK, otherwise known as ripretinib, may be able to help. We will be discussing information about GIST and its treatment, which will include taking a close look at QINLOCK, a prescription medicine used to treat adults with GIST who have received 3 or more prior therapies for their disease.

Today, I am joined by Sandra Brackert, who is a nurse practitioner in the UCLA Sarcoma Program. She has extensive experience in taking care of patients with GIST and sarcomas.

Sandra Brackert, NP: Hi, my name is Sandra Brackert, and I’m a nurse practitioner at UCLA in the Sarcoma Program. I’ve been there since 2008, and we specialize in treating patients with GIST, or gastrointestinal stromal tumors. Some of you may go to what’s called a gastroenterologist for treatment, but at UCLA, in the Sarcoma Program, we specialize in treating GIST patients.

Arun Singh, MD: My name is Arun Singh. I’m a medical oncologist at UCLA. I specialize in taking care of patients with GIST and sarcomas. Today, we’re going to discuss some of the basics of GIST, and we’re going to learn a little bit more about this disease and its challenges.

We’re going to talk about some of the treatment options for advanced GIST. We will also talk about the use of QINLOCK, otherwise known as ripretinib. QINLOCK is a different type of tyrosine kinase inhibitor, or TKI for short, designed to treat adults with advanced GIST who have received 3 or more prior lines of treatment for their disease. QINLOCK was approved in 2020.

We’re also going to highlight some of the services and resources to help you on your GIST journey. We’re going to stop periodically to answer some commonly asked questions about this disease and to explain some of the common terms.

It’s important to give you a vocabulary to empower you and to be more knowledgeable about what’s going on with your cancer.

What is GIST?

GIST stands for gastrointestinal stromal tumor. There are about 4,000 to 6,000 cases of GIST per year in the United States; however, the cause of this disease is unknown. There are no known lifestyle factors, such as diet or smoking, that are associated with this disease. The median age of diagnosis is about 60 years old, and it affects men and women approximately equally.

This disease can start anywhere in the GI tract, including the esophagus, stomach, small intestine, colon, and even the rectum. Rarely, GIST can start outside the GI tract, but those are very uncommon situations.

So, how do I evaluate patients with newly diagnosed GIST? There are 2 main things. One, it’s important to test the tumor and look at it under the microscope. It’s important to determine that you actually have GIST and not another type of cancer or sarcoma.

The test will also give us information about how aggressive your GIST is. And this will be used by your oncologist to inform about your treatment. Two, we also need really good imaging to have a clear picture of the tumor and where it is. This will help us determine if the GIST is in 1 location. If it’s in 1 location and it can be removed by surgery, the term for that is “resectable GIST”.

In many cases, GISTs in multiple locations are not resectable. If there are multiple GISTs that affect several organs and if the person has health issues, that can make surgery risky. Also, surgery might not be the best option if the GIST is very large.

Next term is “advanced GIST”. Advanced GIST is defined as GIST that can no longer be treated with surgery alone, or has come back—otherwise known as “recurrent GIST”. We also use the term “metastatic GIST” for GIST that has spread.

GIST is something that you may be living with for a very long time. And, if you have developed advanced GIST, treatment options and needs change over time.

The other thing that’s important are gene mutations and how they impact the outcomes of this disease, as well as the treatment options that are available. That’s what we’re going to discuss next.

Gene mutations and how they affect GIST.

GIST happens when the genes of our body change. Genes make proteins that control how cells work, and mutated genes can cause cells to multiply uncontrollably and become cancerous. Many people have normal genes that we talk about, such as KIT or PDGFRα. And several thousand other genes as well.

However, in GIST, these normal genes, KIT or PDGFRα, have changed or become corrupted, and the term that we use for that change is a “mutation”. These mutations are like “on” switches. It tells a protein called a tyrosine kinase to be on all the time. And this leads to uncontrolled [cell] growth or “proliferations”, the medical term for that, leading to cancer.

We subdivide the mutations into primary or secondary mutations. Primary mutations are the ones that we feel are responsible for starting the growth of GIST tumors. In people with GIST, the most common primary mutations are KIT mutations, in about 70% to 80% of cases.

PDGFRα mutations are found in about 5% to 10% of cases. The other 10% to 15% of mutations are known as wild-type GIST, in which no KIT or PDGFRα mutations are found.

Over time, genes can continue to mutate. These new mutations are called secondary mutations. Secondary mutations can lead to drug resistance. The most common secondary gene mutations in people with GIST are in KIT and PDGFRα, as we’ve mentioned earlier.

Doctors often test for gene mutations found in GIST tumors. Gene mutation testing essentially is taking a piece of your tumor, extracting DNA or RNA from it, and running it on what is called a gene-sequencing machine.

Sometimes we can also do something called “immunohistochemistry”, which is doing special stains on your tumor to see if there are individual proteins present or absent in your particular GIST. Knowing the specific mutation type may help your doctor choose a treatment for you, and it can also help change the dose of the drug that you’re receiving. It’s a really good way to help tailor your treatment.

So, some common questions from people with GIST…

One…

Q: Why did my doctor do my mutation testing twice?

A: There are a few reasons that this may be the case. Over time, your disease can change. That’s because the mutations can change over time. And, for some patients who are developing secondary mutations, it can be important to recheck and to document the new mutations to understand why you may or may not still be responding to therapy.

Also, some patients have multiple deposits of GIST in their abdomen and pelvis. These spots can be different genetically. If you only biopsy one spot, you may not know what’s happening in the other spots, and you only have information on the individual biopsy. Sometimes we can repeat mutation testing if new biopsies are present.

A second question is….

Q: Can different tumors have different mutations?

A: As we discussed, over time, mutations can change; and, for patients who are developing secondary mutations, it can then be important to recheck and to document, to understand why you may or may not still be responding to therapy. The answer is absolutely “yes”—different tumors can have different mutations.

Q: A third question is, the results of my mutation testing included an exon number. What is an exon?

A: An exon is an expressed part of your gene. Further detailed, there are several exons in KIT; for instance, each individual exon can have mutations.

Another way to think about an exon is like a train track or train line. There are different stops on the train line, and the exon number helps to detail exactly where the mutation is.

GIST can be treated in a few ways

GISTs that are in one area and haven't spread are often treated with surgery.

Advanced GIST is GIST that:

  • can no longer be treated with surgery or
  • has come back (recurrent) or
  • has spread (metastatic)

Common treatment options include:

  • Surgery
  • —A type of targeted medicine, including a treatment you may be currently taking.
  • Clinical Trials — Some people with GIST can join clinical trials that test new kinds of medicines
 
Target icon

Fighting advanced GIST with TKI medicines

The current best medicines for advanced GIST are TKIs. They find or target a kind of protein called a tyrosine kinase [TY-ruh-seen KY-nays]. Although TKIs are often used in advanced GIST, it's best to talk to your doctor about what treatment is right for you.

Review the Treatment Options Available for Advanced GIST

Sandra Brackert, NP: OK, let’s talk about treatment options for advanced GIST.

As you just heard Dr. Singh explain, the driving force in GIST are these tyrosine kinases—these proteins that have mutations on them.

There’s a very simple algorithm that we follow for the treatment of GIST, and the first-line treatment is imatinib, the second-line treatment is sunitinib, and the third-line treatment is regorafenib.

Another drug for advanced GIST is QINLOCK, or ripretinib. QINLOCK was recently approved as a fourth-line treatment.

There is another drug you’ll hear about that was also approved around the same time, called avapritinib.

When GIST advances to a certain point, unfortunately that’s a situation where you cannot remove it, and so we need a treatment that’s going to control the disease.

You’ll hear your care team use the words “stable disease”, or “progression-free survival”. This is actually good news. When we can find a treatment that keeps the disease from spreading to new areas, or the tumor itself from growing, or new tumors from popping up, we call it stable disease. That is when we know that we have control of the disease. Progression-free survival, or PFS, is the length of time, during and after cancer treatment, that a patient lives with the disease, but it does not get worse; that is, the tumor does not grow and the cancer does not spread to another part of the body.

We also want to make sure that the treatment is not worse than the disease. We want to make sure that we manage all your symptoms and side effects while you are on these treatments, so that you have the best quality of life, as well.

Q: So we get questions all the time, like what kind of side effects should I expect with TKIs?

A: Well, tyrosine kinase inhibitors may have some similar side effects, but each drug has its own unique set of side effects, and also, each person has their own unique side effects.

We really want you to share with your health care team whenever you get symptoms or have side effects because, quite honestly, we learn from you. A lot of the remedies and solutions that I’ll offer are based on what has and hasn’t worked for my other patients.

 
Mutation icon

About Mutations and Testing

GIST happens when certain genes in the body change.

GIST cells have genes that have changed in some way. These changes are called (mew‑tay‑shuns).

 

In people with GIST, the most common gene mutations are:

 
GIST Mutations Percentage Chart
 

Switch iconIn GIST, mutations can switch 'on' a kind of protein in the body called a tyrosine . A tyrosine kinase with a mutation is like a light switch that’s stuck in the ‘ON’ position. It sends signals that make cancer cells grow and spread uncontrollably.


Mutation testing for GIST

Sometimes, doctors can do a special test for certain gene mutations found in GIST.

The tests can show:

  • If you have or
  • The location of the mutation (exon)

Knowing your mutation type may help your doctors choose a treatment that works best for you.

Why treatments might stop working or someone might have to try a different treatment


Sometimes, genes can continue to change (mutate). This means that someone with GIST can have new and different mutations over time. If that happens, a treatment could stop working, even if it worked before. This is called drug resistance, and it could lead to progression.

Drug resistance means that:

  • Some people do not respond, or treatment stops working—even if it worked before
  • The dose of the current treatment may need to be increased
  • The person has to change to a new or different treatment

Progression means that:

  • Cancer cells may be growing
  • The tumor may be getting bigger and may be spreading

Sometimes certain side effects can make it difficult for someone to stay on a treatment. This is called treatment intolerance, and it may cause the person to have to try a new or different treatment.

Your GIST Journey: Support and Resources

Sandra Brackert, NP: You’re not alone in this journey with GIST; there are plenty of resources to help you. You’ve probably already been involved with most of them, but just to name some, there’s GISTTogether, which is actually a Deciphera-sponsored website, so you might get some new information there that you haven’t seen before. And, of course, there’s The Life Raft Group, and GIST Support International are all good resources and have a lot of great information and support for you.

Also, QINLOCK has a QINLOCK.com, and you can get more information there. If you have questions and if you want to talk to your oncologist about QINLOCK, you can refer them to this website, and they can get a lot of information, as well.

Unfortunately, I know finances and co-pays are always a problem in our health care system. They’re frequently major sources of stress. Deciphera does have a resource for you called AccessPoint. I usually have patients fill out the AccessPoint form at the same time that I send out the prescription.

What this does is it can provide some financial help. Sometimes the co-pay is too high and you may be eligible for assistance. Sometimes it takes too long to get the prescription through, and AccessPoint may be able to supply a short course of QINLOCK to get things started. They can also help with insurance questions, like whether your plan covers QINLOCK, or how much you might have to pay out-of-pocket.

Whether you have Medicare, or private insurance, or no insurance, you definitely want to make sure that you check out AccessPoint. They can help you get financial assistance that you're eligible for, or help you get medication to you because, really, we want to make sure that you get the best drug for you, for your disease, and we don’t want financial problems to be the reason that you cannot get it. So please, please use Deciphera AccessPoint for help.

“When you have exhausted all possibilities, remember this: You haven’t.” I say this because we see now that we have a treatment that works a little differently than the prior tyrosine kinase inhibitors, and it is a really great option for people with advanced GIST who have gone through several other lines of treatment.

I thank you all very much for your time. I hope we have given you some insight into another therapy and have answered any of your questions, and that you know there are resources out there for you and a drug that may be able to benefit you. Thank you very much.

Arun Singh, MD: I want to thank everyone for listening today, and we also were pleased to share this information about QINLOCK with you. And we think that this is a great addition to the armamentarium for treating patients with advanced GIST. Thank you.

Other sources of GIST support

You and your care partners may sometimes feel alone on this journey. But, you’re not alone. Support is available. These resources have a lot of great information that can help you and your care partners through your GIST journey.

You'll have access to information about topics such as:

  • GIST and treatment options, including clinical trials
  • Questions to ask your doctor and nurses
  • Diet and nutrition
  • Connecting with other people with GIST

GIST support and information:

GISTTogether.com (A Deciphera-sponsored website)

GIST Support International

Life Raft Group

GIST Cancer Awareness Foundation

Deciphera is not affiliated with these organizations, does not endorse any particular service or group, and is not responsible for the content on their website or any services or materials they may provide.

Insurance help

Trying to understand your insurance can be a big challenge. Deciphera AccessPoint™ is here to help you make sense of it and find out what help is available.

VISIT DECIPHERA ACCESSPOINT

Explore more

Caution icon Side Effects

Learn about possible side effects with Qinlock.

More info
Heart lock icon How Qinlock Can Help

If it's time for a new treatment, learn how Qinlock can help to treat advanced GIST.

See how
Tablet icon Getting Started

You can take Qinlock with or without food.

Start here

Important Safety Information

Qinlock may cause serious side effects, including:

A skin problem called palmar‑plantar erythrodysesthesia syndrome. Skin problems are common and sometimes can be severe. Tell your healthcare provider right away if you develop redness, pain, blisters, bleeding, or swelling on the palms of your hands or soles of your feet, or severe rash during treatment with Qinlock.

New skin cancers. Qinlock may cause skin cancers called cutaneous squamous cell carcinoma or melanoma. Talk to your healthcare provider about your risk for these cancers. Your healthcare provider should check your skin before and during treatment with Qinlock to look for any new skin cancers.

Check your skin and tell your healthcare provider right away about any skin changes, including a:

  • new wart
  • skin sore or reddish bump that bleeds or does not heal
  • change in size or color of a mole

High blood pressure (Hypertension). High blood pressure is common with Qinlock and can be severe. Your healthcare provider should check your blood pressure regularly during treatment with Qinlock.

Heart problems. Your healthcare provider should check you for signs or symptoms of heart failure before starting Qinlock and regularly during treatment with Qinlock. Heart failure can be serious and can sometimes lead to death. Tell your healthcare provider if you have any of the following symptoms during your treatment with Qinlock:

  • tiredness
  • shortness of breath
  • swelling of your stomach‑area (abdomen), legs or ankles
  • protruding neck veins

Risk of wound healing problems. Wounds may not heal well during treatment with Qinlock. Tell your healthcare provider if you plan to have any surgery before or during treatment with Qinlock. Your healthcare provider should tell you when to stop taking Qinlock before a planned surgery and when you may start taking Qinlock again after surgery.

The most common side effects of Qinlock include:

  • hair thinning or hair loss
  • tiredness
  • nausea
  • abdominal pain
  • constipation
  • muscle pain
  • diarrhea
  • decreased appetite
  • vomiting

These are not all the possible side effects of Qinlock.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1‑800‑FDA‑1088

It is not known if Qinlock is safe and effective in children.

Before taking Qinlock, tell your healthcare provider about all of your medical conditions, including if you:

  • had a type of skin problem called palmar‑plantar erythrodysesthesia syndrome
  • have heart problems
  • have high blood pressure
  • had or plan to have surgery

For females, tell your healthcare provider if you:

  • are pregnant or plan to become pregnant. Qinlock may harm your unborn baby.
  • can become pregnant as your healthcare provider will do a pregnancy test before you start treatment with Qinlock.
  • become pregnant or think you may be pregnant during treatment with Qinlock.
  • can become pregnant. Use effective birth control (contraception) during treatment with Qinlock and for at least 1 week after the final dose. Talk to your healthcare provider about birth control methods that may be right for you.
  • are breastfeeding or plan to breastfeed. It is not known if Qinlock passes into your breast milk. Do not breastfeed during treatment with Qinlock and for at least 1 week after your final dose.

For males with female partners who are able to become pregnant:

  • use effective birth control during treatment with Qinlock and for at least 1 week after the final dose.
  • if your female partner becomes pregnant during your treatment with Qinlock, tell your healthcare provider right away.

Qinlock may affect fertility in males which may affect the ability to have children. Talk to your healthcare provider if this is a concern for you.

Tell your healthcare provider about all the medicines you take, including prescription and over‑the‑counter medicines, vitamins, and herbal supplements. Qinlock and certain other medicines can affect each other causing side effects or affect how Qinlock works.

Know the medicines you take. Keep a list of them to show to your healthcare provider and pharmacist when you get a new medicine.

General information about the safe and effective use of Qinlock.

Medicines are sometimes prescribed for purposes other than those listed in a Patient Information leaflet. Do not use Qinlock for a condition for which it was not prescribed. Do not give Qinlock to other people, even if they have the same symptoms that you have. It may harm them. You can ask your pharmacist or healthcare provider for information about Qinlock that is written for health professionals.

Indication

What is Qinlock?

Qinlock is a prescription medicine used to treat adults with advanced gastrointestinal stromal tumor (GIST) who have received 3 or more prior treatments for their GIST.

Please see complete Prescribing Information, including Patient Information.

Important Safety Information

Qinlock may cause serious side effects, including:

A skin problem called palmar‑plantar erythrodysesthesia syndrome. Skin problems are common and sometimes can be severe. Tell your healthcare provider right away if you develop redness, pain, blisters, bleeding, or swelling on the palms of your hands or soles of your feet, or severe rash during treatment with Qinlock.

New skin cancers. Qinlock may cause skin cancers called cutaneous squamous cell carcinoma or melanoma. Talk to your healthcare provider about your risk for these cancers. Your healthcare provider should check your skin before and during treatment with Qinlock to look for any new skin cancers.

Check your skin and tell your healthcare provider right away about any skin changes, including a:

  • new wart
  • skin sore or reddish bump that bleeds or does not heal
  • change in size or color of a mole

High blood pressure (Hypertension). High blood pressure is common with Qinlock and can be severe. Your healthcare provider should check your blood pressure regularly during treatment with Qinlock.

Heart problems. Your healthcare provider should check you for signs or symptoms of heart failure before starting Qinlock and regularly during treatment with Qinlock. Heart failure can be serious and can sometimes lead to death. Tell your healthcare provider if you have any of the following symptoms during your treatment with Qinlock:

  • tiredness
  • shortness of breath
  • swelling of your stomach‑area (abdomen), legs or ankles
  • protruding neck veins

Risk of wound healing problems. Wounds may not heal well during treatment with Qinlock. Tell your healthcare provider if you plan to have any surgery before or during treatment with Qinlock. Your healthcare provider should tell you when to stop taking Qinlock before a planned surgery and when you may start taking Qinlock again after surgery.

The most common side effects of Qinlock include:

  • hair thinning or hair loss
  • tiredness
  • nausea
  • abdominal pain
  • constipation
  • muscle pain
  • diarrhea
  • decreased appetite
  • vomiting

These are not all the possible side effects of Qinlock.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1‑800‑FDA‑1088

It is not known if Qinlock is safe and effective in children.

Before taking Qinlock, tell your healthcare provider about all of your medical conditions, including if you:

  • had a type of skin problem called palmar‑plantar erythrodysesthesia syndrome
  • have heart problems
  • have high blood pressure
  • had or plan to have surgery

For females, tell your healthcare provider if you:

  • are pregnant or plan to become pregnant. Qinlock may harm your unborn baby.
  • can become pregnant as your healthcare provider will do a pregnancy test before you start treatment with Qinlock.
  • become pregnant or think you may be pregnant during treatment with Qinlock.
  • can become pregnant. Use effective birth control (contraception) during treatment with Qinlock and for at least 1 week after the final dose. Talk to your healthcare provider about birth control methods that may be right for you.
  • are breastfeeding or plan to breastfeed. It is not known if Qinlock passes into your breast milk. Do not breastfeed during treatment with Qinlock and for at least 1 week after your final dose.

For males with female partners who are able to become pregnant:

  • use effective birth control during treatment with Qinlock and for at least 1 week after the final dose.
  • if your female partner becomes pregnant during your treatment with Qinlock, tell your healthcare provider right away.

Qinlock may affect fertility in males which may affect the ability to have children. Talk to your healthcare provider if this is a concern for you.

Tell your healthcare provider about all the medicines you take, including prescription and over‑the‑counter medicines, vitamins, and herbal supplements. Qinlock and certain other medicines can affect each other causing side effects or affect how Qinlock works.

Know the medicines you take. Keep a list of them to show to your healthcare provider and pharmacist when you get a new medicine.

General information about the safe and effective use of Qinlock.

Medicines are sometimes prescribed for purposes other than those listed in a Patient Information leaflet. Do not use Qinlock for a condition for which it was not prescribed. Do not give Qinlock to other people, even if they have the same symptoms that you have. It may harm them. You can ask your pharmacist or healthcare provider for information about Qinlock that is written for health professionals.

Indication

What is Qinlock?

Qinlock is a prescription medicine used to treat adults with advanced gastrointestinal stromal tumor (GIST) who have received 3 or more prior treatments for their GIST.

Please see complete Prescribing Information, including Patient Information.