GCUK Patient Meetings - 2007 Manchester Reports

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

GIST Cancer UK Meeting , 21st May 2007, Manchester

We had a most interesting meeting, attended by around 80 GIST patients and their carers. We were fortunate enough to be joined for part of the day by Dr Mike Leahy and Dr Bill Newman from the Christie Hospital in Manchester. We are very grateful to them for spending their time with us and for fielding a wealth of questions.

We are also grateful to Professor Garry Whitelam for minuting the meeting and for preparing the following report, which has also been checked by Dr Leahy and Dr Newman.

Speakers & Session Reports

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Roger Wilson - The formation of the Sarcoma Trust

The morning session of the meeting began with some opening remarks from Roger Wilson (Sarcoma UK), Judith Robinson and David Cook.

Roger highlighted the following recent developments:

  • The formation of The Sarcoma Trust which aims to become a registered charity. The Trust was formed in January and has applied for registration with the Charities Commission. The Trust will become a focus for fund raising.
  • Team Sarcoma UK will be holding an awareness event in Torquay in July, as part of a worldwide sarcoma awareness initiative.
  • Roger also asked whether the GIST Cancer UK would support joining the European Cancer Patient Coalition, a lobby group that includes Sarcoma UK as a member organisation. There was widespread support for joining.
  • On the issue of lobbying, Roger reported that the UK Government, in the form of The All Party Parliamentary Group on Cancer (chaired by Dr Ian Gibson, MP), are investigating a cancer reform strategy, "A New Vision for Cancer", launched in March 2006, to replace the "NHS Cancer Plan".

Judith highlighted the GIST Cancer UK mailing list. Those interested in joining should contact David Cook or David Robinson (contact details are available on the GIST Cancer UK website).

David Cook informed the meeting that a member is about to undergo Hepatic Artery Embolisation (HAE) as a treatment for liver metastasis.

Dr Mike Leahy - Q&A Session on Treatment

The remainder of the morning session involved a free question and answer session with Dr Michael Leahy (Consultant Medical Oncologist, Christie Hospital, Manchester).

Question: How could intolerance to bread occur following gastrectomy?
Answer: The stomach is extremely important to the functioning of the whole GI tract. With time the bowel may be able to adapt to some extent.

Question: Is there a National Centre of Excellence for GIST?
Answer: There are clear guidelines for the follow-up after the successful complete surgical resection of a primary GIST and this may not require referral to a specialist team, although expert pathological review to ensure a correct diagnosis is crucial. The management of Imatinib (Glivec) treatment does require special expertise and there are currently about eight centres with large enough practices to develop such expertise. A re-organisation of services for patients with sarcoma is currently under way in the UK. There are currently 34 Cancer Networks, but not all have sarcoma teams. The plan is to join Cancer Networks together into super-regional zones and to ensure that each of these has sarcoma specialists. In the meantime, every patient has the right to request a second opinion from a suitable specialist.

Question: What are the long term effects of Imatinib (Glivec)?
Answer: This is largely unknown because there are still relatively few patients who have been on the drug for more than a few years. A recent study has suggested that Imatinib (Glivec) may display cardiotoxicity (effects on the heart), although such cases seem to be very rare. The Christie Hospital has begun performing routine cardiac monitoring of Imatinib (Glivec) patients as a precaution.

Question: Is it possible that Imatinib (Glivec)-resistant tumours could become responsive after a break from the drug?
Answer: Additional mutations are frequently detected in the c-KIT gene of Imatinib (Glivec)-resistant tumours. In theory, Imatinib (Glivec) withdrawal could lead to competition between tumour cells carrying multiple mutations and those carrying single mutations (i.e. those that may be Imatinib (Glivec)-sensitive). However, the potential benefits of Imatinib (Glivec) withdrawal are doubtful.

Question: What is the recommended action for management of paediatric GIST following resection?
Answer: Childhood GIST is very rare and generally not sensitive to Imatinib (Glivec). It is possible that Sunitinib (Sutent) may prove be useful in cases of recurrence.

Question: Could the appearance of lung nodule represent GIST metastasis?
Answer: The lung is not usually a primary site of GIST metastasis. Lung metastases can develop, but usually after metastases have developed in the abdomen and pelvic regions.

Question: For patients that have tried both Imatinib (Glivec) and Sunitinib (Sutent) what treatment options are available?
Answer: Imatinib (Glivec) is tyrosine kinase inhibitor with quite a narrow spectrum of activity, whereas Sunitinib (Sutent) has a wider spectrum of activity, Sunitinib (Sutent) which is therefore called a multi-targeting kinase inhibitor. This has the potential additional benefit that it can affect angiogenesis (the development of new blood vessels to tumours). Nilotinib is a new kinase inhibitor which is highly potent but has a narrow spectrum of activity like imatinib and is currently undergoing trials. Some new combination therapies are also in trials.

Question: What is the optimal frequency of CT scans following resection of primary GIST?
Answer: There is no clear evidence of the optimal post-operative scanning schedule. The guidelines we are mostly using are a consensus of expert opinion only. It is possible to predict the risk of relapse after surgery from the tumour size and mitotic index (cell division rate). Based on this, the consensus is to scan higher risk patients more frequently. For patients who have had a high risk tumour removed, the guidelines recommend scans every 6 months for 3 years then annually to 5 years after surgery. The guidelines recommend Imatinib (Glivec) treatment immediately upon recurrence. It is not known whether giving Imatinib (Glivec) immediately after surgery (adjuvant therapy) would be beneficial or harmful. A trial of this regime is underway in Europe and Christie Hospital and Royal Marsden are involved in this trial.

Question: What is the importance/availability of GIST tumour genotyping?
Answer: Mutation status can be useful in determining an appropriate therapy/patient management strategy, e.g. 400mg Imatinib (Glivec) versus 800mg of Imatinib (Glivec) for some of the rarer variants.

Question: Would cases of paediatric GIST have preference when PCT budgets are squeezed?
Answer: Paediatric cases are generally well prioritised.

Question: What may be the benefits of surgery for metastases in Imatinib (Glivec) patients?
Answer: The value of this type of surgery in largely unknown and a trial is being organised but may be difficult to complete. Meanwhile, several centres are performing surgery for secondaries when they are confined to the liver and some centres are also offering surgery for secondary peritoneal disease.

Question: Is neutropenia (reduced count of a type of white blood cell) a potentially severe side-effect of Imatinib (Glivec)?
Answer: Some reduction in blood count is common, but cases where this requires withdrawal of Imatinib (Glivec) are very rare.

Question: What trials of new drugs are underway?
Answer: Currently, c-KIT and PDGFRα are the primary targets of drug development. For example, AZD2171 (a multi-targetting tyrosine kinase inhibitor) is undergoing Phase II trials at the Christie. In the future other components of the signalling cascade downstream of these kinases may also become targets. RAD001 targets mTOR which is one of the downstream targets and is being used in combination therapy trials. Other potential targets for therapy include proteins such as bcl2, which is involved in preventing tumour cell death.

Question: Is there a recommended diet for GIST patients?
Answer: GI tract surgery often forces a change in diet. The side effects of Imatinib (Glivec) (e.g. nausea, diarrhoea) may also require a change in diet. No specific diet is known that reduces the risk of relapse.

Question: How important is the width of margins during resection of primary GIST?
Answer: Primary GISTs tend to be very localised, especially in the stomach. Clean margins are more important than wide margins.

Question: Are MRI scans preferable to CT scans?
Answer: MRI scans tend to be better for visualising liver metastases, whereas CT scans are better for visualising peritoneal metastases. Routine surveillance for relapse commonly uses CT, which gives a good liver view and a better peritoneal view.

Question: How long should Imatinib (Glivec) be continued following resection of secondary sites?
Answer: We really don't know. The current view is that, for most patients, treatment should be given indefinitely.

Question: What can be done to manage ascites (fluid accumulation on the peritoneal cavity) following liver resection?
Answer: This is rare and should be monitored.

Question: Are PCT decisions on drugs only an issue of funding?
Answer: NICE determines whether drugs give value for money. Sunitinib (Sutent), although licensed, is not yet approved by NICE.

Dr Bill Newman - Q&A session on mutational testing

The afternoon session began with Dr Bill Newman, a consultant clinical geneticist at Manchester University, introducing clinical genetics and describing some of his research interests. He then took part in a question and answer session.

Question: Can mutation status tests be applied to archived tissue?
Answer: This is difficult, but is a focus of current work and there has been some success. Many hospitals do retain samples.

Question: Are other centres in the UK carrying out mutation status testing?
Answer: There are other research laboratories carrying out these tests, but Manchester is offering this as a clinical service. In mainland Europe there is a major centre for GIST genotyping in Leuven (Belgium) that has been helpful in establishing the service in Manchester.

Question: Will the service be affordable?
Answer: In Belgium, cancer diagnosis entitles patients to have 2 genetic tests performed. In the UK discussion about how testing could be funded are ongoing. The genetic test costs around £300, which is small compared with the costs of drug therapy. It is hoped that in the future the results of genotyping will be used in clinical management.

Question: Should all GIST patients request a mutation status test?
Answer: The standard immunohistochemistry test (for KIT-positive cells) is fine for diagnosis in most cases. The genetic test may help with determining drug dosage and with predicting outcomes. A European study has been completed which indicates some benefits in genetic testing but further studies are required before such testing becomes routine

Question: Does genetic testing help the patient?
Answer: At the moment genetic tests have relatively little direct impact on individual patient care. However, in the future this type of testing may have an increased role to play in prognosis and management.

Question: Is there evidence for inheritance of GIST?
Answer: Familial GIST does exist, but it is extremely rare. Almost all GISTs involve mutations of genes within the tumour itself and NOT throughout the body. Since germ cells (eggs and sperm) do not carry the mutations, typical GIST is not inherited.

Question: Can GIST be transferred from a pregnant mother to a developing foetus?
Answer: There is no evidence that this can happen.

Business Meeting

The final session of the meeting involved a discussion of GIST Cancer UK business.

GIST Cancer UK structure

Judith pointed out that the size of the meeting indicated that the GIST Cancer UK website is clearly working. Judith reminded the meeting of the MailTalk service.

GIST Cancer UK currently does not have a formal structure and its finances are handled by Sarcoma UK. Judith asked whether we need more structure, with appointed officers, such as a treasurer and secretary etc. There was some discussion of the possible benefits of a more formal structure, but no consensus was reached. There was good support for the idea of actual membership, perhaps with an annual subscription. This should be considered after attainment of charitable status by The Sarcoma Trust.

Prototype cards for GIST patients

Feedback was requested about the design of cards that could be carried providing details of medication and names of consultants etc. Two prototypes were available to view. In the same vein, a draft document, to allow travel with medications, was to be posted for comments/feedback.

Next meeting

The next meeting of GIST Cancer UK will be in London, at the Royal Institution of British Architects (66 Portland Place), on October 25th 2007. It was agreed to invite a complementary therapist to attend.

Posted: 2/6/2007