Gastrointestinal Stromal Tumour (GIST)

Gastrointestinal stromal tumours (GISTs) are uncommon tumours of the gastrointestinal (GI) tract, with only 1 person in 100 000 people per year suffering from GIST. These tumours start in very early forms of special cells in the wall of the gastrointestinal (GI) tract - also known as the digestive tract - called the interstitial cells of Cajal (ICCs). ICCs are cells of the autonomic nervous system, the part of the nervous system that regulates body processes such as digesting food. ICCs are sometimes called the “pacemakers” of the GI tract because they signal the muscles in the GI tract to contract to move food and liquid along by an action called peristalsis.

More than half of GISTs start in the stomach. Most of the others start in the small intestine, but GISTs can start anywhere along the GI tract. A small number of GISTs start outside the GI tract in nearby areas such as the omentum (an apron-like layer of fatty tissue that hangs over the organs in the abdomen) or the peritoneum (the layer of tissue that lines the organs and walls of the abdomen).

Some GISTs seem to be much more likely to grow into other areas or spread to other parts of the body than others. Doctors look at certain factors to help tell whether a GIST is likely to grow and spread quickly, such as how large the tumour is, where it's located in the GI tract, and how fast the tumour cells are dividing (its mitotic rate, described in Tests for Gastrointestinal Stromal Tumours).

This only covers clinically relevant GISTs, since, if investigated, a much higher number of lesions 1 cm in diameter (microGISTs) can be found at histopathological examination of stomach tissue in middle-aged and elderly individuals. There is a slight predominance in males. The median age of diagnosis is around 60-65 years, with a wide range. Occurrence in children is very rare.

cancer family: Rare Adult Solid Tumours

A solid tumour is an abnormal mass of tissue that usually does not contain cysts or liquid areas. Solid tumours may be benign (not cancerous), or malignant (cancerous). Different types of solid tumours are named for the type of cells that form them.

Rare adult solid tumours include rare skin cancers and non-cutaneous melanoma, rare thoracic tumours, rare female genital cancers, neuroendocrine tumours, endocrine gland tumours, sarcomas, digestive rare cancers, rare urological and male genital tumours, brain and central nervous system neoplasms, and head and neck cancers.

Approved treatment by the EMA

  • Imatinib 
  • Sunitinib 
  • Regorafenib 

With varying degree of reimbursement, these treatments are reimbursed in several Member States.

How to use the data

Please note that the data available are only meant to inform you on the availability of a specific treatment in European Union Member States and the United Kingdom. However, the treatment might only be available in specific stages or settings of the disease. Please contact your doctor to discuss any questions you might have.

(The information above was obtained during the year 2018, the latest update took place in February 2020.) 

PLease CLICK ON a country to find out about treatments and EUROPEAN REFERENCE NETWORKS (ERN) CENTERS OF EXCELLENCE IN THIS COUNTRY.
Austria0
Belgium4
Bulgaria0
Croatia0
Cyprus0
Czech Republic2
Denmark1
Estonia0
Finland1
France7
Germany6
Greece0
Hungary1
Ireland0
Italy17
Latvia0
Lithuania1
Luxembourg0
Malta0
Poland1
Portugal3
Romania0
Slovakia0
Slovenia1
Spain3
Sweden2
The Netherlands8
United Kingdom7

More useful information

Definition of European Reference Networks:

European Reference Networks for rare, low prevalence and complex diseases (ERNs) are virtual networks involving healthcare providers across Europe. They aim to facilitate discussion on complex or rare diseases and conditions that require highly specialised treatment, and concentrated knowledge and resources.

For more information on Gastrointestinal stromal tumours (GISTs), please visit the following websites:

Patient organisations

Other useful links and information