Gastrointestinal stromal tumours (GIST)- Pathology and clinical applications of recent molecular advances --A perspective review.

Authors : Sharmila DUDANİ, Shivani KALHAN, Sonia SHARMA, Anshu GUPTA

Pages : 100-90

View : 59 | Download : 13

Publication Date : 2014-09-11

Article Type : Review Paper

Abstract : Gastrointestinal stromal tumours insert ignore into journalissuearticles values( GISTs); are the most common   mesenchymal tumours of the GI tract. They occur most frequently in stomach insert ignore into journalissuearticles values(60-70%);, or small intestineinsert ignore into journalissuearticles values(SI); insert ignore into journalissuearticles values( 25-30%);. Rare sites include colon, rectum, appendix and esophagus insert ignore into journalissuearticles values(<10%);. On histology their appearance varies from cellular spindle cell tumours to epithelioid to pleomorphic tumours. Traditionally, three criteria have been used to determine malignancy, which include site of origin insert ignore into journalissuearticles values(SI and rectal tumours   are more aggressive than stomach tumours);, tumour sizeinsert ignore into journalissuearticles values( >5 cm); and mitotic rate.insert ignore into journalissuearticles values(>5/50 hpf );.GISTs are characterized by gain of function mutation in c kit insert ignore into journalissuearticles values(CD117); proto oncogene most commonly involving exon 11. GISTs without c kit mutation show mutations in   platelet derived growth factor receptor alphainsert ignore into journalissuearticles values( PDGFRα);.Surgery has been the standard treatment for resectable GISTs, but   metastatic and unresectable GISTs had a poor prognosis. Recent molecular advances have opened new vistas leading to the development of specific molecular targeted therapies which stabilize the disease and reduce the frequency of disease recurrence. This review summarizes our existing knowledge, recent advances regarding histogenesis, pathology, molecular biology, and targeted cancer therapies which has revolutionized the management of these diseases.    
Keywords : Gastrointestinal stromal tumours, c kit

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