PAWS GIST

Some background

There are some sub-variants of GIST which are extremely rare and which have  been grouped together as "PAWS-GIST".

These are:

Paediatric/Adolescent
Wild-Type
Syndromic (Carney's Triad and Carney-Stratakis Syndrome)

Paediatric/Adolescent GIST differs from adult GIST in many respects. It is more prevalent among girls and young women than among boys and young men. In the main, the illness starts in the stomach, where several primary tumours can present themselves at the same time. Metastases in the lymph nodes are also commoner than in the case of ordinary GIST.

If the genetic make-up of the tumour cells is examined (mutation analysis), typically no mutations are found in either of the genes which are frequently altered in adult patients (KIT and PDGFRA), and this is referred to as "Wild-Type GIST".

Imatinib seems to work less effectively on patients with PAWS-GIST than it does for ordinary GIST. But there is also 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 are often found to have the wild-type GIST.

Syndromic GIST A few paediatric/adolescent GIST patients may also have what is known as "Carney's Triad" or the " Carney-Stratakis-Syndrome". In both these illnesses, further tumours are found along with the GISTs. In cases of Carney-Stratakis-Syndrome there are also tumours originating in the nervous system. These are called paragangliomas. In patients with Carney-Triad, benign lung tumours (pulmonary chondromas) are 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 just suffering initially from paediatric/adolescent 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.

PAWS-GIST Clinic

An alliance of medical specialists and patients is working to help further knowledge on these rare kinds of GIST.

Since work started it has helped to develop the infrastructure for GIST research in the UK and in addition to establishing a PAWS-GIST clinic www.pawsgistclinic.org.uk which convenes three times per annum to develop expertise in the treatment of these types of GIST and to provide specialist support for patients.  It has been the catalyst for the National GIST Biobank and a variety of research projects including work to establish Wild-type GIST cell lines and Xenografts.

Get involved

Because PAWS-GISTs are so rare we still know relatively little about them. However, there are some centres where studies are being carried out, notably in Bethesda in the USA and also via the PAWS-GIST clinic at Addenbrookes Hospital in Cambridge UK.

If you are a Paediatric or Wild-type GIST patient/carer, please make contact with us at: info@pawsgistclinic.org.uk. Our numbers are small, so we need everyone to be involved in order to take full advantage of the help that is being offered.