Current management of gastrointestinal stromal tumors--a comprehensive review

Gastrointestinal stromal tumors (GISTs) comprise < 1% of all gastrointestinal (GI) tumors, but GISTs are the most common mesenchymal tumors of the GI tract. Dramatic changes in clinical practice have been observed in the last decade. This review highlights the overall management of GIST and its recent developments.

We identified literature by searching Medline and PubMed from January 1995 to December 2011 using the keywords "gastrointestinal stromal tumors", "GIST", "imatinib" and "tyrosine kinase inhibitor". Additional papers were identified by a manual search of the references from the key articles. There were no exclusion criteria for published information to the topics.

For localized primary GISTs, surgical resection is the mainstay of therapy. The 5-year survival rate after complete resection of GISTs is approximately 50%-65%. Many factors including tumor size, mitotic rate, tumor location, kinase mutational status and occurrence of tumor rupture have been extensively studied and proposed to be predictors of survival outcomes. Adjuvant imatinib is proposed as an option for those patients with a substantial risk of relapse. Unresectable metastatic or recurrent GIST can be treated with a tyrosine kinase inhibitor, imatinib, with a remarkable response (50%-70%) and prolonged survival (median progression-free survival: 18-20 months; median overall survival: 51-57 months). The standard approach in the case of tumor progression on 400 mg once per day is to increase the imatinib dose to 400 mg twice per day as permitted by toxicity. Use of a second-line targeted agent, sunitinib, in patients with advanced GIST who fail (or are intolerant of) imatinib therapy is advised.

Treatment for GISTs has become increasingly complex because of the growing understanding of its biology. A multidisciplinary team that includes radiologists, medical oncologists, pathologists, and surgeons is paramount for the effective treatment of GIST.