Official websites use. Share sensitive information only on official, secure websites. Corresponding Author: Ronald P. Gastrointestinal stromal tumor GIST is a rare neoplasm that has recently become an intense focus of scientific investigation, as it serves as a model for the molecular therapy for cancer. Multimodality therapy integrating surgery and molecular therapy has shown promise. In this review, we will summarize the epidemiology, clinicopathologic features, natural history, and clinical management of GIST.
The literature on gastrointestinal stromal tumors GISTs has expanded exponentially, demonstrating how medical advancement in the understanding of a disease can revolutionize its diagnosis and treatment. In the past, these tumors were classified as leiomyomas, leiomyosarcomas or leiomyoblastomas. Only recently it has become evident that GIST is a separate entity and the most common sarcoma of the gastrointestinal GI tract.
GIST is a mesenchymal tumor that mainly arises from the alimentary tract but can also develop in the omentum and mesentry. The term GIST was first coined in by Mazur and Clark to describe GI non-epithelial neoplasms that lacked immunohistochemical features of Schwann cells, and did not encompass ultrastructural characteristics of smooth muscle cells. In , Hirota et al.
The intracytoplasmic portion of KIT functions as a tyrosine kinase. In , Joensuu et al. The dramatic response achieved in a solid tumor with targeted agent imatinib has made GIST a paradigm for the use of new molecular agents.
GISTs demonstrate a fairly equal distribution between men and woman, although some literature suggests that GIST has a slight male predominance. Although GIST has been documented in patients of all ages, most of the people affected by GIST are between 40—80 years old at the time of diagnosis, with the median age of 60 years.
