Review
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World J Gastrointest Oncol. Jun 15, 2013; 5(6): 102-112
Published online Jun 15, 2013. doi: 10.4251/wjgo.v5.i6.102
A gist of gastrointestinal stromal tumors: A review
Ashwin Rammohan, Jeswanth Sathyanesan, Kamalakannan Rajendran, Anbalagan Pitchaimuthu, Senthil-Kumar Perumal, UP Srinivasan, Ravi Ramasamy, Ravichandran Palaniappan, Manoharan Govindan
Ashwin Rammohan, Jeswanth Sathyanesan, Kamalakannan Rajendran, Anbalagan Pitchaimuthu, Senthil-Kumar Perumal, UP Srinivasan, Ravi Ramasamy, Ravichandran Palaniappan, Manoharan Govindan, The Institute of Surgical Gastroenterology and Liver Transplantation, Centre for GI Bleed, Division of HPB Diseases, Stanley Medical College Hospital, Chennai 600001, India
Author contributions: Rammohan A, Sathyanesan J, Rajendran K, Pitchaimuthu A and Perumal SK contributed to the conception and design, acquisition, analysis and interpretation of data; Rammohan A, Sathyanesan J, Srinivasan UP and Ramasamy R drafted the article and revised it critically for important intellectual content; Sathyanesan J, Palaniappan R and Govindan M gave the final approval of the version to be published.
Correspondence to: Dr. Ashwin Rammohan, The Institute of Surgical Gastroenterology and Liver Transplantation, Centre for GI Bleed, Division of HPB Diseases, Stanley Medical College Hospital, Old Jail Road, Chennai 600001, India. ashwinrammohan@gmail.com
Telephone: +91-988-4173583 Fax: +91-44-25289595
Received: March 31, 2013
Revised: June 1, 2013
Accepted: June 8, 2013
Published online: June 15, 2013
Processing time: 100 Days and 23.5 Hours
Abstract

Gastrointestinal stromal tumors (GISTs) have been recognized as a biologically distinctive tumor type, different from smooth muscle and neural tumors of the gastrointestinal tract (GIT). They constitute the majority of gastrointestinal mesenchymal tumors of the GIT and are known to be refractory to conventional chemotherapy or radiation. They are defined and diagnosed by the expression of a proto-oncogene protein detected by immunohistochemistry which serves as a crucial diagnostic and therapeutic target. The identification of these mutations has resulted in a better understanding of their oncogenic mechanisms. The remarkable antitumor effects of the molecular inhibitor imatinib have necessitated accurate diagnosis of GIST and their distinction from other gastrointestinal mesenchymal tumors. Both traditional and minimally invasive surgery are used to remove these tumors with minimal morbidity and excellent perioperative outcomes. The revolutionary use of specific, molecularly-targeted therapies, such as imatinib mesylate, reduces the frequency of disease recurrence when used as an adjuvant following complete resection. Neoadjuvant treatment with these agents appears to stabilize disease in the majority of patients and may reduce the extent of surgical resection required for subsequent complete tumor removal. The important interplay between the molecular genetics of GIST and responses to targeted therapeutics serves as a model for the study of targeted therapies in other solid tumors. This review summarizes our current knowledge and recent advances regarding the histogenesis, pathology, molecular biology, the basis for the novel targeted cancer therapy and current evidence based management of these unique tumors.

Keywords: Gastrointestinal stromal tumors; c-KIT; Imatinib mesylate; Surgery; Review

Core tip: Gastrointestinal stromal tumors have been recognized as a biologically distinctive tumor type, different from smooth muscle and neural tumors of the gastrointestinal tract. This review summarizes our current knowledge and recent advances regarding the histogenesis, pathology, molecular biology, the basis for the novel targeted cancer therapy and current evidence based management of these unique tumors.