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Unusual Cancers of Childhood (PDQ®): Treatment - Patient Information [NCI] - Unusual Cancers of the Abdomen



Treatment for carcinoid tumors in the appendix in children may include the following:

  • Surgery to remove the appendix, when the tumor is small and only in the appendix.
  • Surgery to remove the appendix, lymph nodes, and part of the large intestine, when the tumor is larger, has spread to nearby lymph nodes, and is in the appendix.
  • Surgery, chemotherapy, and/or radiation therapy for tumors that have spread.

Treatment for carcinoid tumors that have spread to the large intestine or stomach is the same as treatment for colorectal cancer.

For tumors that make hormones that cause symptoms, medicine can be given to help relieve the symptoms.

See the PDQ summary on adult Gastrointestinal Carcinoid Tumors Treatment for more information.

Gastrointestinal Stromal Tumors

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.

Risk Factors and Symptoms

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. Symptoms of anemia include the following:

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

Other conditions that are not anemia caused by GIST may cause these same 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 and check nearby lymph nodes for signs of cancer. If cancer is in the lymph nodes, the lymph nodes are removed.
  • Watchful waiting for tumors that come back in the same place or cannot be removed, but do not cause symptoms.

WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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