Gastrointestinal Stromal Tumors (GISTs)

What are Gastrointestinal Stromal Tumors?

Initiating in the digestive system, gastrointestinal stromal tumors (GISTs) account for less than one percent of GI tumors. GISTs are often found in the small intestine or stomach, although such tumors may develop anywhere within the GI tract. Not all GISTs are cancerous, although testing will need to be done to make a determination, with results dictating available treatment options.

What are the Symptoms of Gastrointestinal Stromal Tumors?

Signs of Gastrointestinal Stromal Tumors

The exact cause of GISTs isn’t known. The tumors develop early in the gastrointestinal tract in special cells found within GI tract walls that help regulate digestive processes. Some patients will have no initial discomfort from GISTs unless the tumor causes an obstruction or grows to a point where it’s affecting the GI tract. Related symptoms are specific to location.

Signs of GISTs may include

  • Non-specific abdominal pain
  • Noticeable abdominal bulging
  • Feeling of “abdominal fullness”
  • Unexplained fatigue or a general sense of not feeling well
  • Signs of an infection (peritonitis) if the GI tract was penetrated

Gastrointestinal Stromal Tumors Causes

The exact origin of gastrointestinal stromal tumors (GISTs) isn’t known, but a common link to the disease is hereditary. They develop in the stomach or the upper part of the small intestine. In some instances, these tumors are random with no association to genetics, lifestyles or the environment.

Individuals with a family history of this disorder have a higher risk of developing multiple tumors compared to no family history dealing with a single incident.

Two genes recognized with this condition are proto-oncogene tyrosine-protein kinase (KIT) and platelet-derived growth factor receptor, alpha polypeptide (PDGFRA). Both belong to a family of genes responsible for producing proteins vital to cellular growth. Alterations in either of the genes obstruct its role; creating a mutation, instigating uncontrolled cell growth contributing to the tumors.

GISTs are more prevalent in girls than in boys, linked to another gene called SDHB, capable of suppressing abnormal growth in one of four modules of the succinate dehydrogenase (SDH) enzyme. SDH is involved in developing the inside cell formation by converting energy from the foods we consume. An SDH deficiency prompts the abnormal growth of cells leading to tumors.

How are Gastrointestinal Stromal Tumors Treated?

A combination of tests are typically needed to confirm or rule out GISTs. Aside from a complete blood count, testing may involve serum chemistry studies, liver function tests, image tests, a barium swallow test or enema, and a biopsy.

Treatment includes

For GISTs that cannot be removed surgically, therapy drugs called tyrosine kinase inhibitors (TKIs) are sometimes used to block signals tumors need to continue to develop. Treatment may also include chemotherapy and radiation therapy.

Most gastrointestinal stromal tumors are small, typically less than 4 centimeters in diameter. As with other neuroendocrine tumors (tumors that form in cells), GISTs are considered rare. GISTs are often detected during endoscopic testing for suspected GI problems or during surgery to treat an obstruction, hemorrhage, or other issue. The American Cancer Society generally recommends preventative screenings once a year.

Gastrointestinal Stromal Tumors Prevention

Preventative methods to lessen the risk of GISTs do exist. Although you can’t change genetics, you can watch your health with specific behavior modifications to prevent tumors. If a family history of this condition exists, understanding more about your own family genetics is an important health practice. Educate your family since this condition puts future generations at risk.

Early detection is always the best prevention. It’s key to visit your doctor to monitor your health status. Lifestyle changes from maintaining a healthy weight to staying active and eliminating harmful habits like smoking and excessive alcohol can reduce the risk of GISTs.