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).
60%—Stomach.
4%—Duodenum.
30%—Jejunum/ileum.
<1%—Colon (leiomyoma/sarcoma more common).
<<1%—Appendix.
2–4%—Rectum/anorectum.

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 ?
Gastrointestinal stromal tumour of the stomach showing lymph node metastases.
Gastrointestinal stromal tumours of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with longterm follow-up.
Gastrointestinal pacemaker cell tumour (GIPACT): gastrointestinal stromal tumours show phenotypic characteristics of the interstitial cells of Cajal.
Best Cases from the AFIP: Gastrointestinal Stromal Tumour of the Small Bowel.
Gastrointestinal stromal tumours (GIST) are neoplastic lesions of the gastrointestinal tract that commonly arise in the stomach and are being diagnosed with increasing frequency.
Characteristics of KIT-negative gastrointestinal stromal tumours and diagnostic utility of protein kinase C theta immunostaining.
SUTENT is also approved for both gastrointestinal stromal tumours (GIST) after disease progression on or intolerance to imatinib mesylate, and advanced renal cell carcinoma (RCC) in over 100 countries and has been studied in over 10,000 patients in clinical trials.
PDGFR expression in differential diagnosis between KIT-negative gastrointestinal stromal tumours and other primary soft-tissue tumours of the gastrointestinal tract.
INTRODUCTION: Gastrointestinal stromal tumours (GISTs), previously termed as leiomyomas and leiomyosarcomas are relatively common tumours of the gastrointestinal tract occurring in up to 46% of stomachs in some post-mortem series [1].
Gastrointestinal stromal tumours, or GISTs, are pretty rare.
A quality control program for mutation detection in KIT and PDGFRA in gastrointestinal stromal tumours.

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