Gastro-intestinal stromal tumour--role of CD117 in diagnosis and management.

ABSTRACT

Gastro-intestinal stromal tumours (GIST) are a biologically distinct heterogenous group of tumours of the gut. They are said to arise from interstitial cells of Cajal in gut wall. The turnour results from mutation of c-kit gene which codes for CD117 containing tyrosine kinase receptor of Cajal cells. Identification of this mutation by immunohistochemistry (IHC) is the key to the diagnosis of these tumours. CD117 negative GISTs develop from gene mutation through alternate pathway (PDGFRA). The accurate diagnosis is important as specific chemotherapeutic agents are now available for their management. We have studied 8 cases of GISTs during last 2 years in our institute. Half of the cases were female, six cases were in the age group between 35 to 50 years, the other two being of 19 and 70 years. On histology, 5 cases were categorized as high grade on the basis of their size and mitotic count. All cases were subjected to IHC. Only 4 cases were CDll7 positive, one case was positive for S100 and one case for SMA. Remaining 2 cases, negative for CD117, S100 and SMA, histologically resembled GISTs. CD117 positive cases are ideal candidates for treatment with molecularly targeted specific chemotherapeutic agents, e.g., imatinib as these tumours are non-responsive to conventional chemotherapy. Histologically diagnosed stromal tumours of the gut should be subjected to immunostain for CD117 so that specific medical management can be provided to prevent recurrence and metastasis as well as pre-operative debulking of the tumour.