ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumours: Surgery for Small Intestinal and Pancreatic Neuroendocrine Tumours

Abstract

The small intestine and pancreas are among the most frequent abdominal sites of origin of neuroendocrine tumours. Distinctive features of these forms are represented by the relatively low incidence and the wide heterogeneity in biological behaviour. In this light, it is difficult to standardize indications for surgery and the most appropriate approach. It would be helpful for surgeons managing patients with these tumours to have guidelines for surgical treatment of small intestinal neuroendocrine tumours and pancreatic neuroendocrine tumours. The proposed guidelines represent a consensus of the working group of the European Neuroendocrine Tumor Society (ENETS).

Update on Surgical Management of Small Bowel Neuroendocrine Tumors

“…Preoperative tests should include computed tomography (CT) or MRI, somatostatin receptor scintigraphy (SRS) or 68 gallium positron-emission tomography (PET)/CT, 5-hydroxyindoleacetic acid (5-HIAA) levels in 24-h urine sample, and echocardiogram for assessing the burden due to carcinoid syndrome (10). The main scope of this workup is the prevention of perioperative carcinoid crisis presenting with hyperthermia, shock, arrhythmias, hyper-or hypotension, tachy-or bradycardia, flushing or bronchospasm.…”

“…Dissection of at least eight (10,14,20) or 12 (23) lymph nodes has a positive impact on survival. A recent metaanalysis showed that among patients with metastatic disease, palliative resection of the primary tumor conferred a survival benefit (24).…”

“…However, technique standardization and specific guidelines for its role are still under debate (27). Whole small bowel (from Treitz ligand to the ileocecal valve) inspection and palpation with both hands is of great importance for pinpointing otherwise undetectable tumors (10,18). Preoperative CT-or MRI-angiography may be useful in determining the tumor and the lymph nodes sites in relation to the vessels, so that the optimal surgical resection is feasible, with adequate length of small bowel left behind (at least three jejunal arteries) in order to prevent short bowel syndrome (28,29).…”

“…Systematic lymphadenectomy with dissection of at least eight lymph nodes is important and confers a survival benefit (10). Some authors suggest 12 lymph nodes as the cut-off value (28), still others believe that disease recurrence is not predominantly influenced by lymphatic spread but by other factors such as multifocal disease, which should also be taken into consideration (22).…”

“…In this setting, surgery aims to reduce the disease burden in order to improve survival. Patients with operable disease have better prognosis than those with inoperable, even in advanced stages (9,10). Approximately 80% of disease-related deaths are due to liver insufficiency and 16% due to bowel obstruction; therefore, surgical intervention may help improve survival and quality of life (11).…”