gastrointestinal stromal tumour

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gastrointestinal stromal tumour

A mesenchmal tumour consisting of malignant spindled cells, which affects patients above age 50. GISTs are common in the stomach and thought to arise in the interstitial cells of Cajal in the muscularis propria.

Gastrointestinal stromal tumour
1–4%—Oesophagus (leiomyoma more common).
<1%—Colon (leiomyoma/sarcoma more common).

Extragastrointestinal GIST
• Omentum;
• Mesentery.

Pathogenesis GIST
85–90%—c-KIT (tyrosine kinase receptor; activation favours proliferation) mutations.
5%—GISTs PDGFRA (platelet derived growth factor receptor alpha, a c-KIT homologue) mutation.
5%—GISTs Wild type, neither mutation.
Clinical findings
Symptoms are nonspecific and include bleeding, abdominal pain, nausea, vomiting and weight loss.

Adverse prognostic features
Epithelioid type, nuclear atypia, high mitotic count, necrosis, large size, non-gastric location, mucosal invasion, invasion of other organs, metastasis; possible other factors include Ki-67 labelling index, p53 overexpression, p16 loss, KIT mutation, PDGFRA mutation.
References in periodicals archive ?
V600E BRAF mutations are alternative early molecular events in a subset of KIT/PDGFRA wild-type gastrointestinal stromal tumours.
Gastrointestinal stromal tumours (GISTs) negative for KIT (CD117 antigen) immunoreactivity.
In conclusion, we have reported a rare case of a gastric IMT combined with a gastric gastrointestinal stromal tumour and hepatic syringious haemangioma.
Glivec is indicated in the UK for the treatment of adult patients with Kit (CD117) positive unresectable and/or metastatic malignant gastrointestinal stromal tumours (GISTs).
FRIMLEY, Surrey, August 12 /PRNewswire/ -- The National Institute for Clinical Excellence (NICE) has today announced its final decision to fully approve the use of breakthrough drug Glivec (R) for the treatment of gastrointestinal stromal tumours (GISTs), a life-threatening cancer of the GI tract.
Gastrointestinal stromal tumours (GIST) are a rare disease that metastasises in up to 85% of patients with subsequent median progression-free survival (PFS) of only around 30 months.
Sutent has been designed as a treatment for advanced kidney cancer and gastrointestinal stromal tumours (GIST) and is intended for use when other drugs fail.
Kidney cancer affects 6,600 UK patients a year, and 1,000 suffer from gastrointestinal stromal tumours, which Sutent also benefits.

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