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Our data suggests that, in addition to pediatric and adolescent patients, a significant portion of diagnosed "young adults" may also have the pediatric form of GIST.
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Other very rare clinical manifestations of GIST include hyperpigmentation or mastocytosis (both found in inherited forms of GIST) [ 7, 8] and hypoglycemia [ 9- 12].
In the world of big pharma, most eyes are going to be on Pfizer 's drug Sutent, which is being developed for a rare form of stomach cancer called GIST, as well as for cancer of the kidney.
Using the c-kit tyrosine kinase inhibitor, imatinib mesylate, remarkable therapeutic results have been achieved in human malignancies overexpressing a mutated form of c-kit, especially GISTs.
GISTs are the most common form of mesenchymal gastrointestinal neoplasm, but calcification of GISTs is relatively uncommon, with reports in the literature ranging from 3%to25%5%[ 36 38].
Studies are also being conducted to evaluate the drug in treating gastrointestinal (GI) stromal tumors (GIST, a form of sarcoma) [ 5], small-cell lung cancer, prostate cancer, and glioblastoma.
These all form part of a modern, multidisciplinary approach to the management of GIST patients.
A subset of GIST lack specific KIT or PDGFRA mutations and form a heterogeneous group, including NF1, Carney Triad (CT), Carney-Stratakis Syndrome (CSS), pediatric and young adult GIST, and a small proportion (<10%) of sporadic adult GIST [ 3- 8].
Mrs Duff had GIST, a rare form of cancer found in the digestive system, most often in the wall of the stomach, it later emerged.
It appears that, although imatinib treatment induces apoptosis and causes cell cycle arrest of GIST cells, a fraction of the cells usually survive, and these surviving cells may subsequently form the nidus of an imatinib-resistant GIST, often containing secondary KIT mutations (Antonescu et al, 2005; Heinrich et al, 2006).
Occasionally, the industry has scored big hits like Herceptin for breast cancer and Gleevec for chronic myeloid leukemia and GIST, for a rare form of intestinal cancer.
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