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World J Methodol. Sep 20, 2022; 12(5): 381-391
Published online Sep 20, 2022. doi: 10.5662/wjm.v12.i5.381
Diagnosis and management of small bowel neuroendocrine tumors: A state-of-the-art
Jhean Gabriel Gonzáles-Yovera, Pela J Roseboom, Marcio Concepción-Zavaleta, Isamar Gutiérrez-Córdova, Esteban Plasencia-Dueñas, María Quispe-Flores, Anthony Ramos-Yataco, Carlos Alcalde-Loyola, Frederick Massucco-Revoredo, José Paz-Ibarra, Luis Concepción-Urteaga
Jhean Gabriel Gonzáles-Yovera, Division of Gastroenterology, María Auxiliadora Hospital, Lima 15801, Peru
Pela J Roseboom, Division of Emergency Medicine, Regional Academic Hospital of Trujillo, Trujillo 13011, Peru
Marcio Concepción-Zavaleta, Division of Endocrinology, Stella Maris Clinic, Lima 15084, Peru
Isamar Gutiérrez-Córdova, Division of Gastroenterology, Guillermo Almenara Irigoyen National Hospital, Lima 12590, Peru
Esteban Plasencia-Dueñas, María Quispe-Flores, Frederick Massucco-Revoredo, Division of Endocrinology, Guillermo Almenara Irigoyen National Hospital, Lima 12590, Peru
Anthony Ramos-Yataco, Division of Internal Medicine, Ricardo Cruzado Rivarola Hospital, Ica 110301, Peru
Carlos Alcalde-Loyola, School of Medicine, National University of Trujillo, Trujillo 13007, Peru
José Paz-Ibarra, Department of Medicine, School of Medicine, National University of San Marcos, Lima 15001, Peru
Luis Concepción-Urteaga, Division of Internal Medicine, School of Medicine, National University of Trujillo, Trujillo 13011, Peru
Author contributions: Gonzáles-Yovera JG, Roseboom PJ, Concepción-Zavaleta M, and Gutiérrez-Córdova I were the main writers and performed the literature review; Alcalde-Loyola C, Massucco-Revoredo F and Roseboom PJ were translators and prepared the manuscript; Plasencia-Dueñas E, Paz-Ibarra J, Quispe-Flores M, and Ramos-Yataco A performed written contribution to the text body; Massucco-Revoredo F, Paz-Ibarra J and Concepción-Urteaga L performed a general literature review; all authors have read and approved the final manuscript.
Conflict-of-interest statement: All the authors declare no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Anthony Ramos-Yataco, MD, Staff Physician, Division of Internal Medicine, Ricardo Cruzado Rivarola Hospital, Ica 110301, Peru. aj96@outlook.es
Received: March 6, 2022
Peer-review started: March 6, 2022
First decision: April 12, 2022
Revised: April 22, 2022
Accepted: August 5, 2022
Article in press: August 5, 2022
Published online: September 20, 2022
Processing time: 194 Days and 6 Hours
Abstract

This review provides an update on the epidemiology, pathophysiology, symptoms, diagnosis and treatment of neuroendocrine neoplasms (NENs) of the small bowel (SB). These NENs are defined as a group of neoplasms deriving from neuroendocrine cells. NENs are currently the most common primary tumors of the SB, mainly involving the ileum, making the SB the most frequently affected part of the gastrointestinal tract. SB NENs by definition are located between the ligament of Treitz and the ileocecal valve. They are characterized by small size and induce an extensive fibrotic reaction in the small intestine including the mesentery, resulting in narrowing or twisting of the intestine. Clinical manifestations of bowel functionality are related to the precise location of the primary tumor. The majority of them are non-functional NENs and generally asymptomatic; in an advanced stage, NENs present symptoms of mass effect by non-specific abdominal pain or carcinoid syndrome which appears in patients with liver metastasis (around 10%). The main manifestations of the carcinoid syndrome are facial flushing (94%), diarrhea (78%), abdominal cramps (50%), heart valve disease (50%), telangiectasia (25%), wheezing (15%) and edema (19%). Diagnosis is made by imaging or biochemical tests, and the order of request will depend on the initial diagnostic hypothesis, while confirmation will always be histological. All patients with a localized SB NEN with or without near metastasis in the mesentery are recommended for curative resection. Locoregional and distant spread may be susceptible to several therapeutic strategies, such as chemotherapy, somatostatin analogs and palliative resection.

Keywords: Neuroendocrine; Tumor; Small bowel; Small intestine; Gastrointestinal disease; Treatment; Survival

Core Tip: There are reviews in the literature regarding neuroendocrine tumors in the gastrointestinal tract specifically in the small bowel. Nevertheless, this is a first mini review to synthesize the latest data related to epidemiology, pathophysiology, clinical manifestations, diagnosis and treatment of small bowel neuroendocrine tumors.