Gastrointestinal stromal tumors: Do we follow the current guidelines? A self-critique

Abstract

Background: Despite its rarity, gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Several guidelines are currently present where, among other recommendations, mutational analysis and referral to specialized centers have been mentioned. However, this might be difficult to apply at times. Aim: The aim of the study is to explore our experience in the management of GIST. Patients and Methods: Histopathologically-proven GISTs, encountered in our hospital in the period from June 2012 to November 2018, were included in the study. Results: We identified 14 patients, 8 males and 6 females, with a mean age of 58.6 years. Thirteen patients were sporadic GIST, while one was syndromic (associated with neurofibromatosis, multifocal, and arose from the small gut). Twelve patients presented in an emergency situation, while two presented in an elective setting. Thirteen cases were primary localized GISTs and one was metastatic. The organs involved were the stomach in five cases, ileum in four cases, jejunum in two cases, duodenum in two cases, and rectum in one case. In 13 cases, the patient's complaint led to the diagnosis, while in one case, it was discovered incidentally on investigations for another illness. The main clinical features were abdominal pain in five cases, melena and anemia in four cases, hematemesis and melena in one case, rectal bleeding in one case, abdominal pain and mass in one case, intestinal obstruction in one case, and urinary retention and constipation in one case. The mean diameter of the cyst on computed tomography was 8.7 cm. An endoscopic biopsy was performed in six occasions and missed the diagnosis in four of them, whereas percutaneous biopsy was performed in five occasions and was suggestive in two cases and diagnostic in the remaining three. According to a combination of stage, (primary, metastatic, or recurrent) size, risk stratification, mode of presentation, performance status, and comorbidities, treatment was planned. Five patients received surgery only, three patients received surgery followed by imatinib, one patient left to be treated elsewhere, and three patients received surgery to be followed by imatinib but did not show up, one patient received imatinib only, and one patient is still under evaluation. The mean duration of follow-up was 65.7 months, where the disease showed no recurrence in four cases, metastasized to the liver in two cases, and death occurred in two cases, while five cases were lost to follow-up. In this series, no mutational analysis was performed as imatinib was the only drug used, and no referral to specialized centers was done. Conclusion: Surgical resection and kit inhibitors, either alone or in sequence, are the main pillars of treatment of GIST. Risk stratification, in addition to the mode of presentation, the presence or absence of metastasis and comorbidities, dictates which plan to follow. Except for mutational analysis, and referral to specialized centers, our practices are in line with the current guidelines to a reasonable extent.