Surgical Management of Primary Small Bowel NET Presenting Acutely with Obstruction or Perforation

Up to 35% of small bowel neuroendocrine tumors (SBNETs) may present with an acute intra-abdominal complication including obstruction, perforation, bleeding or ischemia and may require emergency surgical treatment in centers not normally accustomed to managing patients with neuroendocrine tumors. These patients may have a known diagnosis of SBNET, be suspected as suffering from SBNET or have SBNET diagnosed as an incidental finding on presenting radiology or postoperative pathology. Perioperative priorities include obtaining both clinical and radiological staging with cross-sectional imaging and clinical examination, screening for the presence of carcinoid syndrome and right-sided cardiac disease and assessment of prognosis. Intraoperatively careful attention should be paid to noting the presence and location of multifocal primary and metastatic disease. Ideally, surgical resection with mesenteric lymph node dissection is the treatment of choice for obstructing and perforating lesions. Extended lymphadenectomy along the SMA, SMV and behind the pancreas should be primarily considered an elective procedure. In unwell patients with advanced disease surgical bypass (jejuno or ileocolic) or proximal defunctioning should be undertaken but, given the excellent long-term survivals in patients with stage IV disease, could be considered bridging procedures to elective resection following formal staging and multidisciplinary review.