Management of Locally Advanced and Unresectable Small Bowel Neuroendocrine Tumours

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SURGICAL SYMPOSIUM CONTRIBUTION

Management of Locally Advanced and Unresectable Small Bowel Neuroendocrine Tumours Jonathan Koea1 on behalf of the Commonwealth Neuroendocrine Tumour Research Collaborative (CommNETs) Surgical Section

Accepted: 7 August 2020 Ó Socie´te´ Internationale de Chirurgie 2020

Abstract Three subtypes of small bowel neuroendocrine tumours (SBNETs) have been described: Type A: SBNET with resectable mesenteric disease that does not involve the mesenteric root; Type B: ‘‘Borderline resectable’’ SBNET presenting with mesenteric nodal metastases and fibrosis adjacent but not encasing the main trunk of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV); and Type C: ‘‘Locally advanced or irresectable’’ SBNET where tumour deposits and fibrosis encase the SMA and SMV. Type C SBNETs are rare and constitute around 5% of patients in reported series, although this may underestimate the prevalence. In these patients, almost all will present with symptoms of intestinal ischemia or obstruction and symptom management should be a primary main focus of treatment. All patients should be carefully staged with cross-sectional imaging and 68 Gadotate positron emission tomography, and discussed at a dedicated neuroendocrine tumour multidisciplinary meeting. Expert surgical review should always be sought as experienced centers have a high rate of successful resection of primary tumours and mesenteric disease. If resection is not feasible, surgical bypass should be considered in patients with a discrete and symptomatic point of obstruction. Non-operative management should emphasize symptomatic treatment with somatostatin analogs, nutritional advice and support and palliative care. Successful neoadjuvant approaches utilizing peptide radionucleide receptor therapy and systemic chemotherapy with everolimus or temazolamide/capecitabine have not been reported.

Introduction Small intestinal neuroendocrine tumours (SBNETS) represent one third to one half of all small bowel tumours and have an annual incidence of 1.05/100,000 population [1]. Early SBNETS develop in a submucosal location and rarely cause symptoms [2]. However, the majority of This article is contribution to the Symposium entitled, Surgical Dilemmas and Challenges for the Operating Surgeon in the Management of Small Bowel Neuroendocrine Tumours: A CommNETs Symposium. & Jonathan Koea [email protected] 1

The Department of Surgery, North Shore Hospital, Private Bag 93503, Takapuna, Auckland 0620, New Zealand

SBNET patients present with metastatic disease (50–70% have lymph node metastases and 25–50% have liver, lung or bone metastases at presentation) [3] although these patients have a relatively favourable 60–70% 5-year survival [4]. A number of contemporary treatment options are now also available to enhance quality of life and extend survival in patients with metastatic SBNET [5]. Localized primary SBNETS are usually treated with small bowel resection and regional lymphadenectomy. However, in patients with advanced lo