Addenbrooke’s in radical bid to beat inherited disease

Two sisters in their early twenties have had their stomachs removed as Addenbrooke’s surgeons bid to protect them from a fatal inherited disease.

Lisa and Ruth Bendle who underwent surgery at the Cambridge hospital are believed to be the first siblings in Britain to have their stomachs removed on the same day. The sisters took the decision because they carry a defective gene that caused fatal stomach cancer in their father, cousin and grandmother, and pre-operative tests had already shown that they were developing the disease.

Richard Hardwick, a specialist upper gastro-intestinal tract surgeon at Addenbrooke’s performed both 2½-hour gastrectomy operations on the same day. He is one member of a large multidisciplinary team based in Cambridge who are involved in an international research project investigating inherited gastric cancer.

"To carry out gastrectomies on two sisters on the same day is I think a first in the country. This was a very difficult situation for them having lost their father to stomach cancer but they were both very brave about having the surgery. A lot of people were involved in the process and the whole team is delighted that the outcome is looking so good for Ruth and Lisa."

To date, about 25 families with Hereditary Diffuse Gastric Cancer (HDGC) have been identified by UK research teams. Only a handful of individuals have had gastrectomies so far.

Hardwick acknowledges that as more patients are identified with the faulty gene apparently healthy people will be forced to make the difficult decision whether to undergo surgery.

“ You are taking very fit, healthy people, telling them they have something wrong with them, and doing a very unpleasant operation when they previously felt perfectly well,” Hardwick said. “The problem is that until we can manipulate genes, the only thing we have to offer is large knife solutions.”

Laboratory examination of the Bendle sister’s stomachs after the operation revealed that cancer had already started to grow but luckily it was still very early and they will be cured. Without surgery it is likely that they would have died of the disease within a year or two.

“ Everyone is telling me I should consider myself lucky, because the other people in our family who had this cancer have all been lost,” said Ruth Bendle, a 20-year-old Surrey University student. “I can’t quite believe what has happened. It was a shock to find I had it and it was so far advanced.”

The sisters’ father died two years ago aged 49. His mother had died at 25 when he was still an infant, and a cousin was killed by the disease at 18.

Ruth and Lisa are taking part in trials run by Cancer Research UK into the genetic causes of a type of stomach cancer called diffuse type gastric cancer in people with a family history of the disease. The trials are run in Cambridge, the UK centre for genetic research, and headed by Professor Carlos Caldas and Dr Rebecca Fitzgerald.

Dr Fitzgerald said: “We are particularly interested in families where three or more members who have been affected by stomach cancer in whom at least one member is younger than 50 years old. When families fulfil the criteria which suggests that there may be an inherited genetic cause for their disease then we enter them onto the familial gastric cancer register which is held in Cambridge with their consent. Genetic testing for the faulty gene called E-cadherin or CDH1 is offered to such individuals.

“This is a big decision for families to take because they have to be able to face the consequences of having a positive test. The options for people who have a mutation are either endoscopy screening to try to detect early signs of cancer or preventative surgery. We are trying to find out more about why alterations in this gene cause cancer so that we can offer alternative treatment to these people and understand more about the causes of the more common types of non-hereditary stomach cancer.”

The register for the trials is run by Sarah Dwerryhouse the study research nurse.

For more information about clinical trials please visit www.cancerhelp.org.uk or contact Sarah Dwerryhouse directly: sd449@medschl.cam.ac.uk

Last updated: 28th November 2006