About Gastrointestinal Stromal Tumors (GIST)

About Gastrointestinal Stromal Tumors (GIST)

At Penn Medicine, patients with gastrointestinal stromal tumors (GISTs) receive their care from a multidisciplinary team of nationally recognized experts in the diagnosis, treatment and research of gastrointestinal cancer.  

Gastrointestinal stromal tumors (GISTs) are a type of soft tissue tumor. GISTs are very rare tumors that start in special cells in the wall of the GI tract, called the interstitial cells of cajal. These cells, often called the “pacemaker cells,” regulate body processes like digestion and send signals to the muscles within the gastrointestinal tract to expand and contract, moving food and liquid through the digestive system.

GISTs can occur anywhere within the digestive tract, but are found most frequently in the stomach or small intestine.

Penn Medicine’s multidisciplinary approach to cancer diagnosis and treatment provides better outcomes and gives patients with GIST access to the most advanced treatment, surgical techniques and clinical trials.

Because navigating a cancer diagnosis and treatment options can be difficult, patients who wish to connect with a cancer specialist at Penn Medicine can speak with a cancer nurse, who can help them make an appointment with the right physician.

To connect with a cancer nurse at Penn Medicine, patients should call 800-789-PENN.

GIST Tumor Risk and Prevention

GIST Tumor Risk and Prevention

Some risk factors for cancer, like age and family history, cannot be prevented. Patients who feel they are at risk may benefit from consulting with a risk assessment specialist within Penn Medicine’s GI genetics program.

GIST Risk Factors

Risk factors affect the chance of developing GISTs. Having a risk factor, or even several, does not mean that someone will get cancer.

Most GISTs are sporadic, meaning they have no association with any specific risk factor. But like many cancers, GISTs occur most in people over the age of 50, and they occur both in men and women.  In rare cases, GISTs have been found in members of the same family suggesting a familial risk.

  • Genetic syndromes. These genetic syndromes may increase the risk for someone developing GISTs.
    • Familial gastrointestinal stromal tumor syndrome. This syndrome happens as a result of an abnormal gene passed from parent to child. People with this syndrome may develop GISTs at a younger age than the tumors usually occur.
    • Type 1 neurofibromatosis (von Recklinghausen’s disease). Another genetic disorder, people with this disease may form benign tumors in their nerves. They also have certain types of tan or brown spots on their skin. People with this disease are at an increased risk of developing GISTs and other cancers. 

GIST Prevention

Because there are no known environmental or behavioral risk factors, there are no recommended preventative guidelines for GIST. People with genetic syndromes that have been found to increase their risk should speak with their physicians about screening for or preventing GISTs.

Risk Assessment for Gastrointestinal Cancer at Penn Medicine

Penn Medicine offers programs for patients who want to determine their risk of developing gastrointestinal cancer through GI genetics program.

These programs offer knowledge about the presence of genetic risk factors for cancer and provide patients with important, sometimes life-saving options.

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GIST Symptoms

GIST Symptoms

Many patients with small GISTs report no symptoms. Large GISTs may cause symptoms similar to those of other tumors in the digestive tract. The most common symptom is gastrointestinal bleeding.

Symptoms of GISTs may include:

  • Problems swallowing
  • Feeling of fullness
  • Abdominal pain
  • Swelling of the abdomen
  • Vomiting and diarrhea
  • Bowel obstruction
  • Weight loss without trying
  • Fatigue
  • Anemia

Sometimes, GISTs can rupture, or cause a gastrointestinal obstruction. In those cases, immediate medical attention is required.

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Staging GIST

Staging GIST

Staging systems provide doctors with a common language for describing tumors. After cancer is first diagnosed, a series of tests are used to determine the extent of the cancer and to see whether it has spread to other parts of the body from where it started. Staging is a way of recording the size and growth of a cancer, and developing the plan for treatment. By understanding the stage of their cancer, patients can make informed decisions about their treatment.

Because there is no standard system, many doctors classify gastrointestinal carcinoid tumors into one of three general stages:

  • Localized spread. The tumor has not spread outside the wall of the primary organ, such as the stomach, colon or intestine.
  • Regional spread. The tumor has spread through the wall of the primary organ and to nearby tissues, such as fat, muscle or lymph nodes.
  • Distant spread. The tumor has spread to tissues or organs far away from the primary organ, such as the liver, bones or lungs.

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GIST Treatment at Penn

GIST Treatment at Penn

After being diagnosed with GIST, patients at the Abramson Cancer Center may be evaluated through the Gastrointestinal Cancer Evaluation Center (GICEC). The center provides patients with expert support and evaluation to discuss treatment options and individualized treatment plans.

Patients with GISTs are treated by a multidisciplinary team of cancer specialists who see more patients with gastrointestinal (GI) cancers in one year than many doctors see in their careers.

Penn Medicine’s GI cancer program has physicians nationally recognized for their expertise in the diagnosis and treatment of cancer.

Penn’s GI cancer treatment team includes:

  • Nationally recognized medical experts with years of experience in diagnosing and treating GI cancer.
  • Nurses with advanced training and experience in caring for patients with gastrointestinal cancer.
  • Oncology Navigation Specialists to help patients navigate the health system.
  • Registered dietitians to provide nutrition counseling.
  • Cancer counselors to provide individual or family counseling for issues many cancer patients face.
  • Rehabilitation therapists who specialize in the diagnosis and treatment of physical effects from cancer and its treatment.

Penn’s GI cancer program is also supported by a robust research program with immediate translation from bench to bedside.

Many treatment options including standard treatments and clinical trials are available for patients with GIST.

Patients at Penn Medicine can benefit from participating in clinical trials that take place at Penn. Patients interested in learning more about clinical trials should speak with someone on their treatment team.

Patient navigation Specialists

Every step of cancer treatment – from cancer diagnosis, to surgery and treatment to forming a survivorship plan – comes with different needs and issues that should be addressed.

Oncology Navigation Specialists at the Abramson Cancer Center are available to make a patient’s experience as seamless as possible. As experts in navigating complex health care situations, patient navigation specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including; provide emotional support, identify resources, and ensure access to information, support services, educational programs and community resources.

Learn more about Oncology Navigation Specialists at the Abramson Cancer Center.

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Diagnosing Gastrointestinal Stromal Tumors (GIST)

An accurate cancer diagnosis from trusted cancer specialists is the first step in getting personalized treatment options to treat gastrointestinal stromal tumors (GISTs).

Patients who choose Penn Medicine’s Abramson Cancer Center, benefit from a multidisciplinary team of cancer specialists including gastroenterologists, radiologists, pathologists and surgeons who work together to provide a diagnosis and treatment plan custom designed for each patient with GISTs.

Cancer specialists at Penn Medicine are highly experienced in using the most advanced techniques for diagnosing gastrointestinal stromal tumors and are actively researching better and more precise ways to detect GISTs.

Navigating a cancer diagnosis and treatment options can be difficult, patients who wish to connect with a cancer specialist at Penn Medicine can speak with a cancer nurse, who can help them make an appointment with the right person. Penn Medicine’s contact center has experienced cancer nurses available and ready to guide patients in finding the cancer specialist right for them.

To connect with a cancer nurse at Penn Medicine, patients should call 800-789-PENN (7366).

Screening for GIST

Screening for GIST

Currently, there are no recommendations for screening the general public for GIST, such as mammograms or colonoscopies. However, people who are at increased risk for GIST should talk to their physicians about anal cancer screening.

GISTs may be found early by chance, such as during another examination like an endoscopy. Rarely, a GIST may be seen on an imaging test like a CT scan is performed for other reasons.

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Diagnostic Tools for GIST

Diagnostic Tools for GIST

Penn Medicine’s multidisciplinary treatment team includes nationally recognized pathologists and radiologists who are experts at finding and diagnosing cancer.

If physicians suspect cancer, they might perform or order one or more of these tests to diagnosis and stage GISTs:

  • Medical history and physical exam. A physician feels the abdomen for swelling, fluid or other changes, and checks for enlarged lymph nodes.
  • Imaging tests. Pictures of the inside of the body can help find out whether a suspicious area might be cancerous, to learn how far cancer may have spread, and to help determine if treatment is working.
    • Upper endoscopy. Endoscopy allows the doctor to see the lining of the upper digestive system with a thin, lighted, flexible tube called an endoscope. The patient may be sedated as the tube is inserted through the mouth, down the esophagus, and into the stomach and small bowel. If an abnormality is found, a biopsy is performed.
    • Endoscopic ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. This procedure is often done at the same time as the upper endoscopy. The endoscopic ultrasound (EUS) can show the extent of tumor wall involvement in the wall of and outside the GI tract, as well as enlarged lymph nodes, which may indicate a tumor or advanced disease. Endoscopic ultrasound also allows aspiration of cells from sites within the GI tract wall as well as from structures outside the GI tract such as lymph nodes. EUS-guided aspiration is usually the least invasive way of obtaining cells for diagnosis.
    • Computed tomography (CT) scan. A CT scan creates a 3-D X-ray of the inside of the body. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan is used to see if the tumor has spread to the liver and to detect carcinoid tumors in lymph nodes behind the abdomen. Sometimes, a contrast medium (dye) is used to provide better detail.
    • Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body before the scan. The tumor absorbs the radioactive substance and the scanner detects the substance to produce images.
    • Magnetic resonance imaging (MRI). Magnetic fields are used to create detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.
    • Colonoscopy. Most colon and rectal cancer is found through a colonoscopy. A colonoscopy is a procedure done under sedation.  The physician places a thin, tube-like instrument with a light and a lens for viewing inside the rectum and colon. The scope may also have a tool to remove polyps or tissue samples, which are checked for cancer in a lab.
    • Double balloon enteroscopy. A technique similar to an upper endoscopy and colonoscopy, double balloon endoscopy has the added benefit of inserting a scope down through the mouth or up from the colon. Two balloons help anchor the scope to the intestine as it inches along so it can travel a greater distance, allowing visualization of most, if not all of the small intestine.
    • Capsule endoscopy. Video capsule endoscopies or "wireless" endoscopies help diagnose small bowel diseases or disorders. Patients swallow a capsule that contains a tiny camera and wear a data recorder. As the capsule makes its way through the digestive system, it transmits up to two pictures per second for up to eight hours. The capsule is excreted normally and painlessly usually within 10 to 48 hours
  • Biopsy. When other tests find a change that is possibly cancer, a sample of the suspicious area is removed and viewed under a microscope. A biopsy is the only way to tell if cancer is really present.
    • Fine-needle aspiration biopsy. Fine-needle aspiration (FNA) is the least invasive method of biopsy and it usually leaves no scar. An injection of local anesthesia numbs the area before the surgeon or radiologist uses a thin needle with a hollow center to remove a sample of cells from the suspicious area. Imaging studies such as ultrasound are used to guide the needle to the right location.
  • Lab tests. Lab tests can help a physician diagnose cancer, determine current organ function, learn how other organs may be affected, and look for signs of reoccurrence.

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Staging GIST

Staging GIST

Staging is a way of describing cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's chance of recovery.

Physicians at Penn Medicine use the TNM staging system:

T Stage

The T stage represents the extent of the primary tumor itself.

  • TX: Primary tumor cannot be assessed
  • T0: No evidence of primary tumor
  • T1-4: Increasing degrees of size, number or invasion of the primary tumor.

N Stage

The N stage represents the degree of the involvement of the lymph nodes.

  • NX: The regional lymph nodes cannot be assessed
  • N0: No evidence of spread to the lymph node
  • N1: Regional lymph nodes are involved with tumor

M Stage

The M stage represents whether or not there is spread of cancer to other parts of the body.

  • M0: No evidence of distant spread of the cancer
  • M1: Evidence of distant spread of the cancer including spread to non-regional lymph node chains

The “staging diagnosis” combines the T, N and M groups into four stages (Stage I-IV); some of which have subtypes (a or b) according to overall tumor characteristics.

Physicians at Penn often classify tumors by whether or not they can be resected, or surgically removed.  There are three ways physicians look at tumors:

  • Localized resectable tumors: Tumor can be completely removed by surgery. This includes most stage I and some stage II cancers in the TNM system.
  • Localized unresectable tumors: Cancers that have not spread to the lymph nodes or distant organs, but cannot be completely removed by surgery are classified as localized unresectable. This includes some early stage cancers, as well as stage IIIA and IIIB cancers in the TNM system.
  • Advanced tumors: Cancers that have spread to lymph nodes or other organs are classified as advanced. These include stage IIIC and stage IV cancers in the TNM system. Most advanced tumors cannot be treated with surgery.

In the case of gastrointestinal stromal tumors, the mitotic rate is also measured. The mitotic rate, described as either low or high, is a measure of how fast the cancer cells are growing and dividing. A low mitotic rate predicts a better outcome.

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Gastrointestinal Stromal Tumor (GIST) Treatment

Following the diagnosis and staging of GISTs, cancer specialists at Penn’s Abramson Cancer Center develop a personalized treatment plan. Penn’s treatment options for GISTs include:

  • Surgery
    • Local excision
    • Low anterior resection
    • Liver resection
    • Radiofrequency ablation
  • Radiation therapy
  • Chemotherapy and biologic therapies
    • Targeted therapies
  • Clinical trials
  • Other treatments
    • Integrative medicine and wellness
    • Palliative care
    • Penn Home Care and Hospice

Because navigating a cancer diagnosis and treatment options can be difficult, patients who wish to connect with a cancer specialist at Penn Medicine can speak with a cancer nurse, who can help them make an appointment with the right physician.

To connect with a cancer nurse at Penn Medicine, patients should call 800-789-PENN (7366).

Surgery for GIST

Surgery for GIST

Many tumors are successfully treated with surgery only. Complete removal of the entire tumor is the standard treatment when possible. When complete removal of the tumor is not possible, surgeons often remove as much of the tumor as possible to provide some relief from symptoms.

Penn Medicine offers patients one of the largest gastrointestinal surgical programs in the United States with a nationally recognized record for high-quality patient care and long-term survival.

Local excision

During this operation, the surgeon removes the primary tumor and a margin of tissue around the primary location. Lymph nodes may also be removed, although lymph node metastasis is rare with GIST, and lymphadenectomy of clinically uninvolved nodes is not necessary. Most localized tumors can be surgically removed through a skin incision, but a rectal carcinoid tumor may be removed using an endoscope.

Low anterior resection

This surgery removes a portion of the upper part of the rectum.

Liver resection

If the tumor has spread to the liver, this surgery removes the cancer from the liver. It often helps relieve or reduce the symptoms of carcinoid syndrome.

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Radiation Therapy for Gastrointestinal Stromal Tumors

Radiation Therapy for Gastrointestinal Stromal Tumors

Typically, gastrointestinal stromal tumors (GISTs) are treated with surgery and medical oncological treatments, including chemotherapy and biological therapies.

Depending on the type of tumor, location and size, radiation may be used. However, its use to treat GISTs is rare.

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Chemotherapy and Biologic Therapies for GIS

Chemotherapy and Biologic Therapies for GIS

Penn Medicine specializes in a team approach to treatment with interdisciplinary care and innovative approaches that use chemotherapy and biologic therapies.

Targeted Therapies

Penn’s medical oncologists are combining personalized care with personalized medicine to create targeted immunotherapies for the treatment of cancer. Targeted immunotherapies such as tyrosine kinase inhibitor (TKI) therapy stimulate the immune system to work harder, allowing it to recognize the difference between healthy cells and cancer cells and working to eliminate those that become cancerous. TKI therapy has significantly increased survival rates.

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Clinical Trials

Clinical Trials

Today, more and more people are surviving cancer. Clinical trials benefit patients with access to breakthrough therapies and treatments. These new advances in cancer treatment are occurring every day at Penn Medicine, giving patients hope that even greater discoveries lie ahead. Through clinical trials:

  • Diagnosing cancer has become more precise.
  • Radiation and surgical techniques have advanced.
  • Medications are more effective.
  • Combinations of medical, surgical and radiation therapy are improving treatment effectiveness and enhancing outcomes.
  • Strategies to address the late effects of cancer and its treatment are improving quality of life.

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Other Treatments for GIST

Other Treatments for GIST

In addition to standard treatments and clinical trials, patients at Penn Medicine may wish to add additional therapies and treatments such as massage therapy, acupuncture and art therapy. These therapies do not have curative intent, and are designed to complement standard treatments, not take their place.

Integrative Medicine and Wellness Programs

At Penn Medicine, integrative medicine and wellness services can supplement traditional cancer treatments such as chemotherapy, surgery and radiation therapy. While conventional medicine plays a critical role in eradicating cancer, integrative medicine and wellness programs offer patients and their families ways to enhance the quality of their lives, minimize or reduce the side effects of cancer and cancer treatment, and promote healing and recovery.

The physicians at Penn Medicine are knowledgeable and supportive of complementary cancer treatments. The cancer team works with patients and families to integrate these supportive programs into the overall care plan, while ensuring the safety and health of patients.

The Abramson Cancer Center’s range of integrative supportive services is designed to help patients cope with the cancer experience and improve their overall sense of well-being. Services include:

The Joan Karnell Cancer Center at Pennsylvania Hospital offers a variety of supportive care programs for patients and families, from diagnosis through survivorship. These programs are available at no cost to the patients treated at Pennsylvania Hospital, and some are open to patients treated elsewhere. These services include social work counseling, nutrition counseling, psychological counseling and spiritual counseling.

The Cancer Appetite and Rehabilitation Clinic focuses on patients with loss of appetite and weight.

The Supportive Care Clinic helps patients manage cancer-related symptoms and goals of care.

Integrative support programs include:

Support groups and educational programs are available at Pennsylvania Hospital throughout the year.

Palliative Care

Palliative care provides medical and non-medical interventions to ease the symptoms of cancer and its treatment. Palliative care includes physical, emotional and spiritual care that can enhance the quality of life for cancer patients.

Palliative care can be used to complement traditional cancer therapies, or to improve quality of life when curative therapies are no longer an option.

Palliative care is an approach to patient care that can be integrated with curative therapies at any point from diagnosis to survivorship or end-of-life care.

Palliative care services include palliative chemotherapy, radiation therapy and surgery as well as psychological counseling, art therapy and support groups for patients and families. The goals of palliative care are to enhance the quality of life for cancer patients and their families, and provide emotional and spiritual support to enhance personal growth.

Palliative care services are offered at the Hospital of the University of Pennsylvania and  Pennsylvania Hospital.

Penn Home Care and Hospice Services

Penn Home Care and Hospice Services offer a full range of home health care needs by partnering three top-level home health care services under one roof:

Penn Home Care and Hospice Services offer an array of specialized therapies and medications for patients with cancer and cancer-related conditions.

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Gastrointestinal Stromal Tumor (GIST) Survivorship

There are more than 12 million cancer survivors living and thriving today as a result of advances in cancer treatment. However, cancer treatments can result in physical, emotional and financial complications long after the therapy is complete. Survivorship programs at Penn Medicine are a distinct phase of gastrointestinal stromal tumor (GIST) care and are designed to help patients' transition from their cancer treatment routine to a post-cancer care lifestyle.

Personalized Survivorship Care

Personalized Survivorship Care

At the end of treatment, GIST patients should schedule a survivorship visit with their oncologist and nurse practitioner. At this time, patients are provided with a summary of the treatment received as well as a plan for follow-up care.

Long-term cancer survivors should speak to their oncologist and nurse practitioner about scheduling a one-time, survivorship-focused consultation with a nurse practitioner or physician. Annual or more frequent follow-up appointments are also available.

Penn cancer providers work one-on-one with patients to develop survivorship care plans. Since every cancer is unique, the plans are tailored to the patients. 

The survivorship care plan includes information on:

  • Potential long-term or late side effects of cancer treatment, the symptoms and treatment.
  • Recommendations for cancer screening for disease recurrence or a new cancer.
  • Psychosocial effects, including relationships and sexuality.
  • Planning follow-up visits.

A survivorship care plan encourages patients to review the information with their health care team and become active participants in their follow-up care.

Penn also offers GIST patients a number of support programs and groups to enhance their survivorship care plans.

Patient navigation Specialists

Every step of cancer treatment – from cancer diagnosis, to surgery and treatment to forming a survivorship plan – comes with different needs and issues that should be addressed.

Oncology Navigation Specialists at the Abramson Cancer Center are available to make a patient’s experience as seamless as possible. As experts in navigating complex health care situations, patient navigation specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including; provide emotional support, identify resources, and ensure access to information, support services, educational programs and community resources.

Learn more about Oncology Navigation Specialists at the Abramson Cancer Center.

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Survivorship Programs at Penn Medicine

Survivorship Programs at Penn Medicine

Penn Medicine’s Living Well After Cancer™ Program is a nationally recognized program that focuses on issues facing cancer survivors. In 2007, the Abramson Cancer Center was designated a LIVESTRONG™ Survivorship Center of Excellence Network.  The Abramson Cancer Center is only one of eight LIVESTRONG centers in the United States, and is the only LIVESTRONG Survivorship Center of Excellence in the Philadelphia region. The program focuses on survivorship, a distinct phase of care.

Prescription for Living: The Cancer Survivorship Program at the Joan Karnell Cancer Center at Pennsylvania Hospital, provides patients with a summary of important information about specific cancer diagnoses and treatments, as well as follow-up information and steps to take towards recovery, supportive care and education to help patients adjust to their lives as cancer survivors.

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Continued Support

Continued Support

Penn offers cancer patients support programs and groups to enhance their survivorship care plans.

The Abramson Cancer Center and Joan Karnell Cancer Center at Pennsylvania Hospital offer a wide range of materials and host many activities that provide education and support to address key areas of concern including survivorship for cancer patients and their loved ones.

Focus On: Gastrointestinal Cancers is a day-long conference that addresses issues for patients with gastrointestinal cancer, as well as their loved ones.

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Survivor Stories

Brian Trainor

It was on January 7th that Penn law student Brian Trainor, a healthy and active 31 year old with no known symptoms had a sudden seizure. Brian was rushed to HUP, where he received a CT scan and MRI, which uncovered a 2.5 centimeter tumor on the left frontal lobe of his brain.




Spotlight

Stand Up to Cancer

Peter O'Dwyer, MD, professor of Hematology-Oncology and program director of Development Therapeutics in the Abramson Cancer Center, was interviewed on NBC10's 10! Show about Penn's work as part of the Stand Up to Cancer Dream Team... Read more

Clinical Trials

Clinical trials are studies to find new ways to prevent, detect and treat cancer. The purpose of each clinical trial is to answer a specific question. Our physicians carefully design these studies to find new ways to improve care and quality of life ... more about clinical trials