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From question on page 1582

The strong immunohistochemical staining with c-kit is in keeping with a malignant epitheliod gastrointestinal stromal tumour (GIST). The tumour was inoperable on computer tomography, demonstrating extensive local spread (including the left lateral segment of the liver). GISTs are rare but nevertheless the commonest mesenchymal tumours to arise in the gastrointestinal tract, with an annual incidence of 10 per million per annum. They are characterised by expression of the receptor tyrosine kinase KIT (Cd117), and this expression is often associated with gain of function mutations. Glivec (ST1571, imatinib mesylate; Novartis Pharmaceuticals, Basel, Switzerland) is a selective tyrosine kinase inhibitor that has proved to be effective in achieving tumour shrinkage in up to 60%, and symptomatic benefit in 80% of patients with metastatic disease. Predicting the clinical behaviour of GIST tumours remains imprecise and survival often depends on the ability to achieve complete resection.

Our patient with a gastric GIST with liver metastases had a complete histological resolution after six months of Glivec therapy. This was confirmed by immunohistological examination after the patient underwent total gastrectomy with D2 radical lymphadenectomy and metastectomy of segment VI of the liver. The previous metastatic liver deposits were mucoid and cystic in appearance with no viable tumour cells and the gastric primary was replaced by hyalinsed fibrous tissue and macrophages with some spindle cells indicating where the tumour had previously been. Immunohistochemistry was performed on all lesions and was completely negative for CD117 and CD34. The response to Glivec treatment is variable, and the location and nature of the KIT oncogenic mutations influence the likelihood of clinical response to various KIT inhibitor therapies.

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