The patient is on six months follow-up receiving oral imatinib 300 mg twice a day. Conclusion GIST was first described by Mazor and Clark (1983) . It Regorafenib mw originates from the interstitial
cells of Cajal (ICC), located in the muscularis propria (myenteric plexus) responsible for triggering smooth selleck chemicals muscle contraction [2, 3]. The basic pathology is an activating mutation (gain in function) of chromosome 4 which codes for c-Kit resulting in uncontrolled proliferation of stem cells that differentiate towards ICC. GIST is sporadic . Familial forms with autosomal dominant inheritance have also been documented [3, 4]. Isolated reports of GIST occurring concomitantly with paraganglioma, pulmonary chondroma, nerofibromatosis, pancreatic neuro-endocrine tumours, burkitt’s lymphoma, osteosarcoma, neuroblastoma and melanoma have been documented . 90% of GIST occurs in adults more than 40 years of age (median age 63 years). There is slight male preponderance . No documented elements indicating any association with geographic location, ethnicity,
race or occupation has been elucidated [4, 5]. The commonest site of GIST is stomach (60-70%) [2, 3]. Jejunum accounts for 10% of all GI tract GIST’s [1, 3]. Sporadic reports of GISTs arising from the omentum, mesentery or retroperitoneum, have been documented but most SU5402 chemical structure of these are metastatic from gastric or intestinal primaries . Extra-GIST has been reported in gall bladder, pancreas, liver and urinary bladder . Presentation is erratic. Seventy percent are symptomatic at presentation, 20% are asymptomatic and 10% are detected at autopsy [5, 6]. Common presentations include abdominal pain, palpable mass, gastro intestinal bleeding, fever, Astemizole anorexia, weight loss and anaemia . Isolated jejunal GIST associated with perforation
and peritonitis is a rare and unique . Perforation is usually attributed to replacement of bowel wall by tumour cells, tumour embolization leading to ischemia, necrosis together with raised intra-luminal pressure [4, 5, 7]. In view of the exophytic nature of the growth, intestinal obstruction occurs due to compression rather than luminal obstruction. As such intetstinal obstruction is a rare occurrence until the tumour attains enormous size. Clinical diagnosis of GIST is based on index of suspicion [6, 7]. Specific diagnostic signs and symptoms are absent. Chronicity is a rule. Acute atypical presentation includes hemorrhage and perforative peritonitis [1–10]. Preoperative imaging modalities like contrast enhanced abdominal computerized tomography (CT) aids in diagnosis . The extent of the tumor, metastases and involvement of other organs can be assessed. A dedicated magnetic resonance imaging (MRI) provides better information than CT in the preoperative staging workup [7, 8]. Endoscopy can diagnose gastric GISTs. Endoscopy demonstrates smooth, mucosa-lined protrusion of the bowel wall which may or may not show signs of bleeding or ulceration.