An Enigmatic Pelvic Tumor

Gastrointestinal stromal tumors (GISTs) have been noticed as a biologically special type of tumor, distinct from previously described neural and smooth muscle tumors of the gastrointestinal tract (GIT). Extra Gastrointestinal stromal tumors (EGISTs) are tumors with immune-histological features overlapping with GISTs but found outside the GI tract in the abdomen with no contact with the gastric or intestinal wall. E-GISTs most frequently arise from the omentum, mesentery, retroperitoneum, or solid organs like the liver and the pancreas. E-GISTs originating in the pelvic cavity and not being associated with the GI tract are very uncommon. Furthermore, these E-GISTs are commonly seen in patients over 50 years of age. Imatinib hasexceptional antitumor effects through molecular inhibition and has necessitated a definite diagnosis of GIST. The crucialinterplay between the molecular genetics of GIST and the response to targeted treatment has served as a pathway for the study of targeted therapies in other solid tumors. This case report and review encapsulates our present knowledge about GIST and recent advances regarding histopathology, molecular biology, the basis for the unique targeted therapy, and present evidence-based management of these unusual tumors.