Gastrointestinal stromal tumor (patient information)
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Gastrointestinal stromal tumor |
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Where to find medical care for Gastrointestinal stromal tumor? |
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Gastrointestinal stromal tumor On the Web |
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Directions to Hospitals Treating Gastrointestinal stromal tumor |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]:Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]
Overview
Gastrointestinal stromal tumor (GIST) is a disease in which abnormal cells form in the tissues of the gastrointestinal tract. The abnormal cells in GISTs may be malignant (cancer) or benign (not cancer). They are most common in the stomach and small intestine but may be found anywhere in or near the GI tract. It is thought that GISTs begin in cells called interstitial cells of Cajal (ICC), which are located in the wall of the GI tract.
What are the symptoms of Gastrointestinal stromal tumor?
Common symptoms of GIST include:
- Blood (either bright red or very dark) in the stool or vomit.
- Pain in the abdomen, which may be severe.
- Feeling very tired.
- Trouble or pain when swallowing.
- Feeling full after only a little food is eaten.
- Sometimes GISTs are smaller than the eraser on top of a pencil. GIST tumors may be found during a procedure that is done for another reason, such as an x-ray or surgery. Some of these small tumors will not grow and cause signs or symptoms or spread to the abdomen or other parts of the body. Doctors do not agree on whether these small tumors should be removed or whether they should be watched to see if they begin to grow.
What causes Gastrointestinal stromal tumor?
Genetic mutations are considered the most identifiable cause of GIST. Around 95% of these mutations are sporadic (isolated) with less than 5% occur as part of hereditary, familial, or idiopathic multi tumor syndromes. Common causes of GIST include mutation in proto-oncogenes such as c-Kit gene and PDGFRA gene. These proto-oncogenes control cell multiplication and mutations in them can lead to excessive and uncontrolled cell multiplication leading to tumor formation. In other cases where the patient do not exhibit the typical mutation in c-Kit and PDGFRA gene, mutations in succinate dehydrogenase (SDH) gene have been reported. Rare genes involved include mutation in BRAF kinase and protein kinase C.
Who is at highest risk?
Age and genetic factors can increase the risk of having a GIST. Individuals in the age group of 50-80 years are believed to be at the highest risk. The risk of GIST is also increased in people with certain genetic syndromes such as Neurofibromatosis type 1, Carney-Stratakis syndrome and Familial gastrointestinal stromal tumor syndrome. In rare cases, GISTs can be found in several members of the same family.
Diagnosis
The following tests and procedures may be used:
- Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Endoscopic ultrasound and biopsy : Endoscopy and ultrasound are used to make an image of the upper GI tract and a biopsy is done. An endoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the mouth and into the esophagus, stomach, and first part of the small intestine. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography. Guided by the sonogram, the doctor removes tissue using a thin, hollow needle. A pathologist views the tissue under a microscope to look for cancer cells.
If cancer is found, the following tests may be done to study the cancer cells:
- Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
- Mitotic rate : A measure of how fast the cancer cells are dividing and growing. The mitotic rate is found by counting the number of cells dividing in a certain amount of cancer tissue.
When to seek urgent medical care?
If you experience either of the following symptoms, seeking urgent medical care as soon as possible.
- Severe abdominal pain- The reason a person with GIST appears to have severe abdominal pain is mostly because of gastric wall perforation. This is the result of cancer invading and perforating the gastric wall. Surgical intervention needs to be done urgently.
- Vomiting a lot of blood- This is the result of the cancer invading the vessels of the gastric wall. An abundant amount of blood loss may lead to shock and death.
Treatment options
For many GIST it is important to know the stage of the cancer in order to plan treatment. However, the treatment of GIST is not based on the stage of the cancer. Treatment is based on whether the tumor can be removed by surgery and if the tumor has spread to other parts of the abdomen or to distant parts of the body. Treatment is based on whether the tumor is:
- Resectable: These tumors can be removed by surgery.
- Unresectable: These tumors cannot be completely removed by surgery.
- Metastatic and recurrent: Metastatic tumors have spread to other parts of the body. Recurrent tumors have recurred (come back) after treatment. Recurrent GISTs may come back in the gastrointestinal tract or in other parts of the body. They are usually found in the abdomen, peritoneum, and/or liver.
- Refractory: These tumors have not gotten better with treatment.
Four types of standard treatment are used
- Surgery: If GIST has not spread and is in a place where surgery can be safely done, the tumor and some of the tissue around it may be removed. Sometimes surgery is done using a laparoscope (a thin, lighted tube) to see inside the body. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope is inserted into one of the incisions. Instruments may be inserted through the same incision or through other incisions to remove organs or tissues.
- Targeted therapy: Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells.
- Tyrosine kinase inhibitors: (TKIs) are targeted therapy drugs that block signals needed for tumors to grow. TKIs may be used to treat GISTs that cannot be removed by surgery or to shrink GISTs so they become small enough to be removed by surgery. Imatinib mesylate and sunitinib are two TKIs used to treat GISTs. TKIs are sometimes given for as long as the tumor does not grow and serious side effects do not occur.
- Watchful waiting: Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change.
- Supportive care: If a GIST gets worse during treatment or there are side effects, supportive care is usually given. The goal of supportive care is to prevent or treat the symptoms of a disease, side effects caused by treatment, and psychological, social, and spiritual problems related to a disease or its treatment. Supportive care helps improve the quality of life of patients who have a serious or life-threatening disease. Radiation therapy is sometimes given as supportive care to relieve pain in patients with large tumors that have spread.
Where to find medical care for Gastrointestinal stromal tumor?
Directions to Hospitals Treating Gastrointestinal stromal tumor
Prevention of Gastrointestinal stromal tumor
Once a patient has been diagnosed with GIST, preventive measures include regular follow ups including physical examination with laboratory and imaging evaluations.
- GISTs are known to present with locally recurrent and distant spread and thus periodic evaluation through a CT scan should be done for early detection of recurrent disease and prevention of future complications (such as tumor hemorrhage). However, there is no consensus on the time interval between assessment.
- Patients with indeterminate or inaccessible lesions can be evaluated with PET scans (positron emission tomography) to identify malignant lesions (cancerous) from benign lesions (non-cancerous).
- A positron emission tomography (PET) scanning can also be used to determine response to therapy and can identify active tumor from dead and inactive scar tissue. This is particularly helpful in patients receiving chemotherapy for incompletely resected, recurrent, or metastatic disease.
What to expect (Outlook/Prognosis)?
The prognosis (chance of recovery) and treatment options depend on the following:
- How quickly the cancer cells are growing and dividing.
- The size of the tumor.
- Where the tumor is in the body.
- Whether the tumor can be completely removed by surgery.
- Whether the tumor has spread to other parts of the body.
Possible complications
Common complications of GIST include:
- Gastrointestinal bleeding
- Intestinal obstruction
- Intestinal perforation
- Surgical complications associated with resection include:
Source
https://www.cancer.gov/types/soft-tissue-sarcoma/patient/gist-treatment-pdq Template:WH Template:WS