Surgical Principles in the Management of Small Bowel Neuroendocrine Tumors

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Neuroendocrine Cancers (JR Strosberg, Section Editor)

Surgical Principles in the Management of Small Bowel Neuroendocrine Tumors Alexandra Gangi, MD1 Daniel A. Anaya, MD2,* Address 1 Division of Surgical Oncology, Department of Surgery, Cedars Sinai Medical Center, Los Angeles, CA, USA *,2 Section of Hepatobiliary Tumors, Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center & Research Institute, 12902 Magnolia Drive, Tampa, FL, 33612, USA Email: [email protected]

* Springer Science+Business Media, LLC, part of Springer Nature 2020

This article is part of the Topical Collection on Neuroendocrine Cancers Keywords Neuroendocrine tumors I Small bowel I Mesenteric mass I Surgical management

Opinion statement Small bowel neuroendocrine tumors (SB NETs) are increasing in frequency and becoming more common in surgical practice. It is often difficult to make the diagnosis of a SB NET at an early stage, as the primary tumor tends to be small and patients are asymptomatic until there is regional or distant metastasis, when they develop abdominal pain, partial obstruction, or bleeding and/or develop carcinoid syndrome. Despite this advanced presentation at the time of diagnosis, patients with metastatic SB NETs, as compared to other gastrointestinal malignancies, have favorable survival, which can be improved by appropriate surgical interventions. With the lack of randomized studies, there is reasonable controversy surrounding the optimal management of patients with SB NETs. As such, treatment of these patients is driven primarily by physician experience and available data based predominantly on retrospective studies. Based on this, current recommendations advocate for patients with SB NETs (localized or metastatic) to be managed at experienced centers by a multidisciplinary team. Eligible patients should undergo surgical resection of primary and regional disease as outlined in this article. Additionally, patients with metastatic disease should be evaluated on a case by case basis to evaluate surgical options that may mitigate bowel symptoms (i.e., pain, intestinal angina, obstruction) and carcinoid symptoms (flushing, diarrhea, hemodynamic instability) and prolong survival. Unlike other gastrointestinal malignancies, aggressive surgical management of these patients, even in the context of unresectable metastatic disease, can improve patients’ symptoms and long-term survival. The principles outlined in this article are geared to guide appropriate management of SB NET patients with improvement in quality of life and overall survival outcomes.

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Curr. Treat. Options in Oncol.

(2020) 21:88

Introduction Neuroendocrine tumors (NET) represent a wide range of neoplasms originating from enterochromaffin cells found across different organs, most commonly arising in the lungs, the small bowel, and the rectum [1, 2•]. During the last decade, there have been changes in the pathologic classification and nomenclature of NET [3•], which had previously been subject to confusion and misclassifica