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Gastrointestinal Stromal Tumor Treatment

Gastrointestinal Stromal Tumor (GIST)

What Is Gastrointestinal Stromal Tumor?

Gastrointestinal Stromal Tumor (GIST) is a type of soft tissue sarcoma that originates in the digestive tract, primarily in the stomach and small intestine. Smaller GISTs often cause no health issues, but larger ones can lead to symptoms such as blood in stool or vomit. GISTs are more common in individuals aged 50 to 70 and are rare before the age of 40. It is an uncommon cancer that begins in special cells located in the wall of the digestive tract.

Approximately 4,000 to 6,000 GIST cases are reported annually in the U.S. Genetic factors can increase the risk, especially in families where genetic mutations are common. Types of GIST include:

  • Pediatric GIST: Affects children and young adults
  • Wild-type GIST: Not caused by cell mutation
  • Familial GIST: Inherited from family members
  • Syndromic GIST: Linked with genetic syndromes

Genetic Syndromes Related to GIST:

  1. Carney Triad (CT): Appears in the stomach and with paragangliomas, usually in females in their 20s.
  2. Neurofibromatosis Type 1 (NF1): Caused by a defect in the NF1 gene, leading to noncancerous tumors in the small intestine.

Symptoms of GIST

  • A palpable abdominal mass
  • Nausea and vomiting
  • Abdominal pain or discomfort
  • Bowel obstruction
  • Blood in the stool
  • Vomiting blood
  • Fatigue from anemia (due to internal bleeding)

Bleeding from the stomach may make vomit appear like coffee grounds and cause black, tarry stools. GISTs may block food passage (obstruction), rupture (causing holes in the GI tract), and can lead to emergency symptoms. They are often discovered during check-ups for unexplained abdominal pain, chronic anemia, or GI bleeding.

Prevention of GIST

Surgery is the most effective way to prevent GIST progression:

  • Tumor removal is successful in 85% of cases.
  • Microscopic analysis determines aggressiveness.
  • Key factors for future treatment decisions:
    1. Mitotic index: Rate of cell division.
    2. Tumor size: Tumors <2 cm are less likely to recur.
    3. Tumor location: Tumors in the stomach are less likely to recur than those in the intestine.

Radiation and chemotherapy are not commonly needed but radiation can help control bleeding. Early-stage tumors can often be removed without harming surrounding organs.

GIST Treatment Procedures

Surgical Options

Goal: Remove as much of the tumor as feasible.

  • Surgery is ideal for tumors >=2 cm or non-metastatic.
  • Surgery is not advised if tumors are in critical areas affecting vital organs.

Types of Surgery:

1. Small GISTs

  • Laparoscopy is used:
    • A small cut is made.
    • A lighted camera guides the surgeon to remove the tumor.

2. Larger GISTs

  • Open surgery may be required.
  • May involve removal of nearby organs:
    • Rectum and anus
    • Stomach
    • Pancreas
    • Intestine

3. Recurrent GISTs

  • May spread to the liver.
  • Targeted therapy is used when surgery is no longer viable.
  • Other treatments:
    • Radiation therapy to relieve pain
    • Thermal ablation
    • Surgery (if possible)
    • Clinical trials

Conclusion

Early diagnosis is critical. Positive outlook and mental health support are equally important. A multimodal therapy approach—including radiological evaluation, clinical and preoperative endoscopy, and biopsy—helps determine the risk of metastasis and optimizes treatment planning.

Frequently Asked Questions

Q. Is GIST a Serious Problem?

A: Small GISTs often cause no issues, but larger GISTs can lead to serious complications such as bleeding and obstruction.

Q. What Kind of Drugs Are Used to Cure GIST?

A:

  1. Ayvakit
  2. Imatinib Mesylate
  3. Avapritinib
  4. Regorafenib
  5. Stivarga
  6. Ripretinib

Q. Does GIST Have to Be Taken Out from the Body?

A: Yes, if the tumor is small, it should be surgically removed along with some surrounding tissue. Lymph node removal is typically unnecessary.

Q. Is GIST Curable or Not?

A: GIST can be cured if diagnosed early. Many patients recover without further treatment.

Q. What Is the Survival Rate of GIST?

A: The overall 5-year survival rate for GIST is 83%. Survival outcomes are improving with advancements in treatment and early detection.

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