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Gastrointestinal stomach tumours (GISTs) are uncommon cancers that start in special cells in the wall of the gastrointestinal (GI) tract, also known as the digestive tract. The GI tract processes food for energy and rids the body of solid waste.
Currently, there are very few known risk factors for gastrointestinal stomach tumours (GISTs).
These tumours can occur in people of any age, but they are rare in people younger than 40 and are most common in people older than 50.
Most GISTs are sporadic (not inherited) and have no clear cause. In rare cases, though, GISTs have been found in several members of the same family. These family members have inherited a gene mutation (change) that can lead to GISTs.
Researchers do not know exactly what causes most gastrointestinal stomach tumours (GISTs). But great progress has been made in learning how certain changes in DNA can cause normal cells to become cancer cells.
DNA is the chemical in our cells that makes up our genes, which control how our cells function. We usually look like our parents because they are the source of our DNA. But DNA affects more than just how we look.
People with a GIST often do not experience any specific symptoms or signs. When symptoms do occur, they may be vague. Or, the cause of a symptom may be a different medical condition that is not a tumour.
People often do not experience any particular symptoms with GIST. It is often discovered during a medical evaluation due to chronic anemia, abdominal pain, or gastrointestinal bleeding, or imaging tests done for another reason.
Doctors use many tests to find or diagnose, a GIST. Your doctor may consider these factors when choosing a diagnostic test:
In addition to a physical examination, the following tests may be used to diagnose GIST or determine the best treatment plan. Not all tests listed below will be used for every person.
If a tumour (also called a mass or nodule) is found, the doctor will also do tests to learn if it is cancerous. For most types of tumours, a biopsy is the only sure way for the doctor to know if an area of the body has cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.
After your diagnostic tests, your doctor will review all of the results with you. If the diagnosis is GIST, some of these results also help the doctor describe the tumour.
Staging is a way of describing where the tumour is located, or if it has spread to another part of the body from where it started. If this happens, it is called metastasis. Doctors may also do tests to learn which treatments could work best.
The stages for gastrointestinal stromal tumours (GIST) range from I (1) through IV (4). As a rule, the lower the number, the less cancer has spread. A higher number, such as stage IV, means cancer has spread more. And within a stage, an earlier letter means a lower stage. Although each person’s cancer experience is unique, cancers with similar stages tend to have a similar outlook and are often treated in much the same way.
There are 2 different stage grouping schemes, depending on where cancer starts:
Stage I
A- The tumour is:
B- The tumour is larger than 5 cm but not more than 10 cm across (T3). Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is low.
Stage II
The tumour is no more than 2 cm across (T1). Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is high.
Stage III
A- The tumour is larger than 5 cm but not more than 10 cm across (T3). Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is high.
B- The tumour is larger than 10 cm across (T4). Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is high.
Stage IV
The tumour is any size (Any T) AND it has spread to nearby lymph nodes (N1). Cancer has not spread to distant sites (M0). The mitotic rate can be low or high.
Stage I
The tumour is:
Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is low.
Stage II
The tumour is larger than 5 cm but not more than 10 cm across (T3). Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is low.
Stage III
A- The tumour is no more than 2 cm across (T1). Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is high.
B- The tumour is larger than 2 cm but not more than 5 cm across (T2). Cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0). The mitotic rate is high.
Stage IV
The tumour is any size (Any T) AND it has spread to nearby lymph nodes (N1). Cancer has not spread to distant sites (M0). The mitotic rate can be low or high.
Treatment for your condition can begin immediately and can include:
Radiation therapy kills cancer cells by delivering high-powered beams of energy, such as X-rays or protons.
Radiation therapy can be provided in the following ways:
Chemotherapy is a medicinal treatment that kills cancer cells by using chemicals. It can be taken as a pill, injected into a vein (intravenously), or both. Chemotherapy is most commonly used to treat undifferentiated pleomorphic sarcoma that returns or spreads to other parts of the body after the first treatment.
Targeted medication treatments are designed to target specific abnormalities found in cancer cells. Targeted medication treatments can kill cancer cells by inhibiting these aberrations. Targeted therapy medications may be coupled with chemotherapy in the treatment of undifferentiated pleomorphic sarcoma.
Surgery is usually the main treatment for gastrointestinal stromal tumours (GISTs) that haven’t spread. The goal of the surgery is to remove all of cancer.
The type of surgery needed depends on the location and size of the tumour.
If the tumour is small, it often can be removed along with a small area of normal tissue around it. This is done through a cut (incision) in the skin. Unlike many other cancers, GISTs almost never spread to the lymph nodes, so removing nearby lymph nodes is usually not needed.
For some small cancers, “keyhole” (laparoscopic) surgery is an option. Instead of making a large incision in the skin to remove the tumour, several small ones are used. The surgeon inserts a thin, lighted tube with a tiny video camera on the end (a laparoscope) through one of them. This lets him or her see inside the belly. Long, thin surgical tools are then used through the other incisions to remove the tumour. Because the incisions are small, patients usually recover more quickly from this type of surgery than from traditional surgery that requires a longer incision.
If the tumour is large or growing into other organs, the surgeon might still be able to remove it entirely. To do this, parts of organs (such as a section of the intestines) might need to be removed. The surgeon might also remove tumours that have spread elsewhere in the abdomen, such as the liver.
Another option for tumours that are large or have grown into nearby areas might be to take the targeted drug imatinib (Gleevec) first, typically for at least several months. This is called neoadjuvant treatment and can often shrink the tumour, making it easier to remove with surgery.
Surgery is not a common treatment for a GIST that has spread (metastasized) to other parts of the body. Targeted therapy drugs are usually the first option for metastatic GISTs. But if there are no more than a few metastatic tumours and they respond well to targeted therapy, some doctors might advise surgery to remove them. No large studies have been done to show how helpful this is, but it might be an option. If your doctor offers this surgery, be sure you understand the goals and possible side effects.
If the tumours are in the liver and would be hard to remove, other options might include different types of local treatments, such as ablation or embolization.
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