Learn About Gastrointestinal Stromal Tumor

What is the definition of Gastrointestinal Stromal Tumor?

A gastrointestinal stromal tumor (GIST) is a type of tumor that occurs in the gastrointestinal tract, most commonly in the stomach or small intestine. This type of tumor is thought to grow from specialized cells found in the gastrointestinal tract called interstitial cells of Cajal (ICCs) or precursors to these cells. Affected individuals can develop one or more tumors. GISTs are usually found in adults between ages 40 and 70; rarely, children and young adults develop this type of tumor.

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What are the causes of Gastrointestinal Stromal Tumor?

Genetic changes in one of several genes are involved in the formation of GISTs. About 80 percent of cases are associated with a mutation in the KIT gene, and about 10 percent of cases are associated with a mutation in the PDGFRA gene. Mutations in the KIT and PDGFRA genes are associated with both familial and sporadic GISTs. Less than 10 percent of cases are SDH-deficient GISTs, which are associated with mutations or other changes in the SDHA, SDHB, SDHC, or SDHD gene. SDH-deficient GIST can be familial or sporadic. A small number of people with a GIST have mutations in other genes.

How prevalent is Gastrointestinal Stromal Tumor?

Approximately 5,000 new cases of GIST are diagnosed in the United States each year. SDH-deficient GIST accounts for about 5 to 7 percent of cases. However, GISTs may be more common than the estimate because small tumors may remain undiagnosed.

Is Gastrointestinal Stromal Tumor an inherited disorder?

Most cases of GIST are sporadic and are not inherited. These cases are associated with a somatic mutation, which is a genetic change that occurs only in the tumor cells and occurs during a person's lifetime.

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What are the latest Gastrointestinal Stromal Tumor Clinical Trials?
A Phase Ib, Open-label, Multi-center Study to Characterize the Safety, Tolerability, and Preliminary Efficacy of TNO155 in Combination With Spartalizumab or Ribociclib in Selected Malignancies

Summary: This study is a Phase Ib, multi-center, open-label study of TNO155 in combination with spartalizumab or ribociclib with a dose escalation part followed by a dose expansion part in adult subjects with advanced solid tumors. These two treatment arms will enroll subjects in parallel to characterize the safety, tolerability, PK, PD and preliminary antitumor activity. The study treatment will be admini...

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DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors

Summary: This phase II trial studies nivolumab and ipilimumab in treating patients with rare tumors. Immunotherapy with monoclonal antibodies, such as nivolumab and ipilimumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread. This trial enrolls participants for the following cohorts based on condition: Epithelial tumors of nasal cavi...

Who are the sources who wrote this article ?

Published Date: July 14, 2021Published By: National Institutes of Health

What are the Latest Advances for Gastrointestinal Stromal Tumor?
A Case of Curative Surgery for Locally Advanced Small Bowel GIST after Imatinib Therapy.
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Might Patients with Metastatic Gastrointestinal Stromal Tumors Benefit from Operative Management? A Population-Based Retrospective Study.