Gastrointestinal stromal tumors (GIST) related emergencies

BACKGROUND: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, accounting for 1-3% of all gastrointestinal malignancies. Throughout the whole length of the gastrointestinal tract, GIST arises most commonly from the stomach followed by small bowel. The clinical presentations of GIST are highly variable according to their site and size. The most frequent symptoms are anemia, weight loss, gastrointestinal bleeding, abdominal pain and massrelated symptoms. Patients may present with acute abdomen, obstruction, perforation or rupture and peritonitis. Surgical resection is the "gold standard" for therapy of GIST. Recently, targeted therapy with inhibitors of tyrosine kinase receptors (imatinib) has been introduced for the management of advanced and metastatic tumors. PURPOSE: The aim of this work is to present the experience of the Gastrointestinal Surgery Unit, Alexandria Faculty of Medicine in the management of patients with GIST related emergencies. PATIENTS AND METHODS: This study was carried out on all patients with gastrointestinal stromal tumors who presented to the Gastrointestinal Surgery Unit, Main Alexandria University Hospital in an emergency situation during the period from January 2005 till December 2012. All patients' data, clinical presentations, radiological and endoscopic data, surgical procedures, complications, and survival data were collected, reviewed and analyzed. After approval of local ethics committee, all patients included in the study were informed well about the procedure and an informed written consent was obtained from every patient before carrying the procedure. RESULTS: Between January 2005 and December 2012; 92 patients (54 males and 38 females) were admitted with different emergency presentations of clinically and radiologically suspected GISTs. The tumors were located in the stomach in 49 patients, in the duodenum in 6 patients, in the small intestine in 27 patients, in the small intestinal mesentery in 4 patients, in the colon in 3 patients and in the rectum in 3 patients. The most frequent presenting symptom was gastrointestinal bleeding in 45 patients. Twenty-six patients presented with intestinal obstruction, 14 patients with intraperitoneal hemorrhage and 7 patients with rupture and peritonitis. Ninety patients were operated upon. Two patients presented with extensive GIST, and were not candidate for surgical treatment. All operated patients underwent surgical resection. Complete macroscopic resection was achieved in 86 patients (95.6%), while 4 patients (4.4%) had incomplete resection. All over 11 patients developed metastases, or recurrence. The 3 and 5-years overall survival rates for all patients, using the Kaplan-Meier actuarial curve, were 92.1% and 81.4% respectively. The 3 and 5-years disease-free survival rates for all patients were 73.2% and 64.5% respectively. CONCLUSION: Although GISTs are uncommon, their incidence is probably increasing especially their emergency presentations. The emergency surgeon must be acquainted with the disease, its emergency presentation and principles of surgery in the presence of GIST tumors. Early diagnosis and treatment would save life of many patients who presented with GIST related emergencies. Surgery is still the gold standard treatment in localized GIST, although the percentage of relapse is not low even after radical surgery. The prognosis is strictly related to size and completeness of surgical resection. We strongly advocate that all patients with a GIST be carefully and regularly followed-up for an indefinite period. The large number of patients in this series is an alarming signal for further studies to elucidate the pathogenesis of this disease.