Gastrointestinal Stromal Tumors in Children

Gastrointestinal stromal cell tumors (GIST) usually begin in cells in the wall of the stomach or intestines. GISTs may be benign (not cancer) or malignant (cancer). Childhood GISTs are more common in girls, and usually appear in the teen years.

GISTs in children are not the same as GISTs in adults. Patients should be seen at centers that specialize in the treatment of GISTs and the tumors should be tested for genetic changes. A small number of children have tumors with genetic changes like those found in adult patients. The risk of GIST is increased by the following genetic disorders:

• Carney triad
• Carney-Stratakis syndrome

Most children with GIST have tumors in the stomach and develop anemia caused by bleeding. Signs and symptoms of anemia include the following:

• Feeling tired
• Dizziness
• A fast or irregular heartbeat
• Shortness of breath
• Pale skin

A lump in the abdomen or a blockage of the intestine (crampy pain in the abdomen, nausea, vomiting, diarrhea, constipation, and swelling of the abdomen) are also signs of GIST. Other conditions that are not anemia caused by GIST may cause these same signs and symptoms.

Treatment for children who have tumors with genetic changes like those found in adult patients is targeted therapy with a tyrosine kinase inhibitor.

Treatment for children whose tumors do not show genetic changes may include the following:

• Surgery to remove the tumor.

Treatment of recurrent GIST in children may include the following:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.