A Gastro-Intestinal Stromal Tumour is rare, but you are not alone!


The following report was written by Stefanie Peyk who is 35 years old, from Berlin. Stefanie is a member of the Executive Committee of "Das Lebenshaus"and has had Paediatric GIST since 1992.


We are very grateful to our member Michael Sayers for translating the above report from the original German

Should you wish to get in contact with Stefanie, please
write to peyk@lebenshauspost.org. (She has excellent English!)

Blue cars or green dinosaurs?

First US consultation for patients with paediatric GIST... it is the middle of June 2008, in the waiting room of a large clinic in Bethesda, Maryland. A young man in his early 20's displays his pressure bandage: "Look, I've been given a bandage with blue cars on it." So what? I've got green dinosaurs," a young woman replies. "Green Dinosaurs? Cool! I want green dinosaurs too!" says the young man. The patterns on plasters and bandages are a sure sign that we are in a children's ward. There is a relaxed atmosphere, none of those present look as if they are suffering.

14 GIST-patients aged between 10 and 35, most of them accompanied by relatives, have come for a special appointment/consultation, a consultation especially for patients with paediatric GIST. I am the only patient from Germany and I am the oldest person here. However, in all probability, I too have paediatric GIST.

Paediatric GIST is an extremely rare sub-variant of GIST, which in many respects differs from adult GIST: paediatric GIST is more prevalent among girls and young women than among boys and young men. Here in the clinic, 10 of the 14 patients are female. In the main, the illness starts in the stomach, where several primary tumours can present themselves in one go. There are more metastases in the lymph nodes than in the case of adults. In cases where the genetic make-up of the tumour cells is examined (so-called mutation analysis), typically no mutations are found in either gene, which are frequently altered in adult patients (KIT and PDGFRA). The doctors then talk about "Wild type GIST". Imatinib seems on the whole to work less effectively on patients with paediatric GIST than it does for adult patients. But there is also some good news: the tumours often grow more slowly and behave in a less aggressive way than their counterparts in adults. Young adults suffering from GIST can either have the paediatric form of GIST or the form more usually found in older adults.

Not all the patients here in the waiting room have paediatric GIST. Patients with Carney-Triad or Carney-Stratakis-Syndrome have also been invited. In both these illnesses, further tumours are found along with the paediatric GISTs. In cases of Carney-Stratakis-Syndrome there are found the so-called paragangliomas, tumours which originate in the nervous system. In patients with Carney-Triad, benign lung tumours (pulmonary chondromas) were observed along with GIST and paragangliomas) as well as, in some isolated cases, benign tumours of the suprarenal gland, (adenomas) and benign tumours of the oesophagus (leiomyomas). Patients suffering only from paediatric GIST may develop these other types of tumour over time. While Carney-Stratakis-Syndrome seems to be hereditary, it is not thought that the same applies to Carney-Triads.

Medical science knows relatively little about these three illnesses: paediatric GIST, Carney-Stratakis-Syndrome and Carney-Triad. With a view to bringing this state of affairs to an end, the 14 patients have come to Bethesda. Bethesda is where the USA's National Institutes of Health (NIH) is based. The NIH is a medical research establishment of the USA's Health Ministry. One of the most recent research projects is to find out more about paediatric GIST and related illnesses. In order to achieve this aim, NIH researchers have got together with the two large American GIST-Patient organisations, Life Raft Group and GSI GIST Support International, as well as with GIST-Specialists in other American research institutes, to organise the first consultation for patients with paediatric GIST.

Here we are examined: measured, weighed, blood taken and they ask us about our family history of diseases. An immediate advantage of the consultation for us patients: we can pester selected specialists with questions - experts, who have already established themselves at the forefront of the studies of our disease. Among the doctors here present are well-known American GIST-experts such as George Demetri, an oncologist from the Dana Farber Cancer Center (Boston), Cristina Antonescu, a pathologist from the Memorial Sloan-Kettering Cancer Center (New York), Michael Laquaglia, a paediatric surgeon from the Memorial Sloan-Kettering Cancer Center (New York) and Constantine Stratakis, a geneticist und endocrinologist from the NIH - the Carney-Stratakis-Syndrome is named after him and his colleague Carney.

And the patients ask: "But I am still so young. Is it not therefore risky, to have a CT scan every 3 months? Doesn't the radiation build up over the years?" or: "I have now been tumour free for over a year. I tolerate Imatinib very badly. Must I still take it, despite this?" Every patient gets half an hour with the whole range of experts, including appointments with psychologists, nutrition advisers, social workers. As we wait, we patients have time to talk to each other and get to know one another. I already know many of the mothers here from the Internet: in the USA there is a Mail system especially for patients with paediatric GIST and those associated with them - comparable with the Das Lebenshaus Mail system and the GIST Support UK Mailtalk. It is lovely for once to meet these people face to face, people with whom I have already shared many sorrows as well as many joys. For us patients, therefore, the consultation is a winner, whatever happens.

But why do the researchers go to all this trouble? OK, so they want to help us. And: they want to build up a medical database with the (protected) information of as many patients with paediatric GIST as possible, and perhaps including information about patients with Wild Type-GIST . This internet based, secure database should, for example, give information about disease progression, the response to or failure of therapy, about pathology and radiology findings. The data from us first 14 patients will form the foundation stone for the database; further consultations are planned. Besides this, the researchers hope that any patients, who could not come personally to Bethesda - such as patients from Europe, put off by the long journey - will make their data available to them for the purposes of research. With the aid of these data, which should regularly be updated, the researchers want to learn more about the causes of paediatric GIST - and they want to find better ways of treating the disease.

The doctors in Bethesda described another possible line of inquiry: recent research results show that a particular receptor, Growth Factor Receptor 1, (IGF-R1 for short), which is similar to insulin, is over-produced in Wild type GISTs, so that it seems to appear with unusual frequency on the tumour cells. Clinical studies with antibodies, which block this receptor in a targeted way, suggest a possible future option for patients with paediatric and Wild type-GIST.

We are all completely bemused by so many new things and so much new information. The pressure bandage with the blue cars and green dinosaurs has long since been put aside. What remains is the confident feeling of being in good hands, and the hope that the researchers here will succeed in their search for an effective treatment for our illness.

Since the consultation in June 2008, the scientists taking part have established the Consortium for Pediatric and Wildtype GIST Research, CPGR. Members of this consortium are:
  The Dana Farber Cancer Institute in Boston
  The Memorial Sloan-Kettering Cancer Center in New York
  The Texas Children's Hospital in Houston
  The National Institute of Health in Bethesda, Maryland.

The research consortium is open to other doctors and scientists interested in Paediatric and Wildtype GIST. The scientists who took part will meet together twice a year at the National Institute of Health.

Posted: 20/10/2008

Clinical Trial - 3 v 5 years adjuvant imatinib recruitment extended until 31st March 2020- trial centre list update here

The next PAWS-GIST clinic is happening on Friday 12th April 2019. Registration is via PAWSGISTCLINIC.ORG.UK 

Our next Patient Meetings are in Leeds on Friday 4th October 2019 and London - 6th March 2020. If you would like to join us please email  to register. We look forward to seeing you...

Rare Cancer Patient Survey - collecting feedback on rare cancer patient experience with clinical trials...

On the 10th October the Scottish Sarcoma network are holding a day called RISE at the Western Park Hotel in Falkirk. The day is dedicated to people with a sarcoma diagnosis and their families to focus on "what matters to me". This poster contains the registration details if you would like to attend. GIST Support UK will be represented at this meeting and we look forward to seeing you. 

A big thank you to Scotmid Co-operative for choosing to support GIST Support UK and the development of our national tissue bank. Read more here


If you missed any of our previous meetings, you can view the speakers' presentations here

Clinical Trial - 3 v 5 years adjuvant imatinib - trial centre list update here

19th August 2017 - Page 19 of the Times GIST report - READ HERE

New UK clinical practice guidelines for the management of gastrointestinal stromal tumours (GIST) published by British Sarcoma Group - NEWS >>>

GSUK's Chair Nic Puntis discusses the BBC's report on the Cancer Drugs Fund on BBC Wiltshire Radio - NEWS>>>.  

Update on GIST clinical trials that are recruiting and in the pipeline....

We have launched our first GIST patient survey and hope to gather a better understanding of the landscape for GIST patient treatment in the UK. The survey closes on 10th September 2016 so if you would like to participate please complete the survey by clicking this link

'ALT' GIST Trial to open in the UK soon! We have been alerted to a new trial that will be opening in the UK in the near future for metastatic GIST patients. Read more on our Trials & Research page 

PAWS-GIST Clinic 7 - Newsletter June 2016

GSUK attends Cancer52 parliamentary reception to raise awareness of rare and less common cancers.

GSUK Research Update 2016 - Read all about it here

NHS England's Sarcoma Service Specification consultation is open until Weds 20th April 2016.       Please register your views by responding directly via this link.                             GSUK's response can be viewed here

Cycle for GSUK! We have four allocated charity places on the RideLondon event for this year and also for 2017 and 2018. Go to our News page to find out more!

The Masitinib Trial is now open.  For further details of this trial, and to find out which centres and clinicians are involved, please go to our Research & Trials page. 

Regorafenib has remained on the NHS England Cancer Drug Fund List for GIST patients! Read more!

Can you help Professor Eamonn Maher with his research Study? If you have had, or have, two or more primary GISTs, or a GIST and another tumour, find out more at the bottom of our research page

PRESS RELEASE - UKTV announces GIST Support UK as its Charity of the Year 2015

TUMOUR BANK UPDATE: "We have been granted approval from the NHS ethics committee for our National GIST Tissue Bank which will store these valuable GIST tumour samples for research. If you are about to have an operation and would like to donate your tissue for research please email  with your contact details. Visit our Retention of tissue page for regular updates.

Scottish Sarcoma Network: Interested in attending their Sarcoma education days or finding out about the services on offer ? Read our News page

Use of human tissue
Do go to our new "Retention of Tissue" page and please sign a consent form!

GSUK funds research!
GSUK provides £20,000 from donated funds to help start a research project at the Royal Marsden. Visit our News page for further information and picture.

Funds being raised for UK PAWS GIST clinic
See our News Page for more information.

NICE approves improved treatment for ascites
The PleurX system allows the patient to drain fluid from the abdominal cavity without having to pay repeated visits to hospital. More information on our News Page.

European Commission approves three years' adjuvant use of Glivec
Visit our News page for more details.

A press release in February claiming the effectiveness of this drug as a replacement for Sunitinib appeared to be premature at the time. However subsequent news may be more encouraging! See our News page for more details.