Systematic Reviews
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 7, 2020; 26(25): 3686-3711
Published online Jul 7, 2020. doi: 10.3748/wjg.v26.i25.3686
Quality of life in patients with gastroenteropancreatic tumours: A systematic literature review
Catherine Watson, Craig William Tallentire, John K Ramage, Rajaventhan Srirajaskanthan, Oscar R Leeuwenkamp, Donna Fountain
Catherine Watson, Craig William Tallentire, Donna Fountain, PHMR Health Economics, Pricing and Reimbursement, London NW1 8XY, United Kingdom
John K Ramage, Rajaventhan Srirajaskanthan, Kings Health Partners NET centre, Kings College Hospital London, London SE5 9RS, United Kingdom
Oscar R Leeuwenkamp, Advanced Accelerator Applications, Geneva 1204, Switzerland
Author contributions: Watson C and Tallentire CW performed the research and analysed the data; they contributed to the manuscript equally; Ramage JK has reviewed the manuscript and offered invaluable contribution based on his disease expertise; Srirajaskanthan R also provided disease expertise; Leeuwenkamp OR and Fountain D designed and supervised the research. All authors contributed to writing the manuscript; all authors read and approved the final manuscript.
Conflict-of-interest statement: All the authors declare that they have no competing interests.
PRISMA 2009 Checklist statement: This study followed PRISMA guidelines.
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:
Corresponding author: Donna Fountain, PhD, Research Scientist, PHMR Health Economics, Pricing and Reimbursement, PHMR Head Office, London NW1 8XY, United Kingdom.
Received: March 9, 2020
Peer-review started: March 9, 2020
First decision: March 31, 2020
Revised: June 4, 2020
Accepted: June 17, 2020
Article in press: June 17, 2020
Published online: July 7, 2020

Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) are slow-growing cancers that arise from diffuse endocrine cells in the gastrointestinal tract (GI-NETs) or the pancreas (P-NETs). They are relatively uncommon, accounting for 2% of all gastrointestinal malignancies. The usual treatment options in advanced GEP-NET patients with metastatic disease include chemotherapy, biological therapies, and peptide receptor radionuclide therapy. Understanding the impact of treatment on GEP-NET patients is paramount given the nature of the disease. Health-related quality of life (HRQoL) is increasingly important as a concept reflecting the patients’ perspective in conjunction with the disease presentation, severity and treatment.


To conduct a systematic literature review to identify literature reporting HRQoL data in patients with GEP-NETs between January 1985 and November 2019.


The PRISMA guiding principles were applied. MEDLINE, Embase and the Cochrane library were searched. Data extracted from the publications included type of study, patient population data (mid-gut/hind-gut/GI-NET/P-NET), sample size, intervention/comparators, HRQoL instruments, average and data spread of overall and sub-scores, and follow-up time for data collection.


Forty-three publications met the inclusion criteria. The heterogeneous nature of the different study populations was evident; the percentage of female participants ranged between 30%-60%, whilst average age ranged from 53.8 to 67.0 years. Eight studies investigated GI-NET patients only, six studies focused exclusively on P-NET patients and the remaining studies involved both patient populations or did not report the location of the primary tumour. The most commonly used instrument was the European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-C30 (n = 28) with consistent results across studies; the GI-NET-specific module Quality of Life Questionnaire-GINET21 was used in six of these studies. A number of randomised trials demonstrated no HRQoL changes between active treatment and placebo arms. The Phase III NETTER-1 study provides the best data available for advanced GEP-NET patients; it shows that peptide receptor radionuclide therapy can significantly improve GEP-NET patients’ HRQoL.


HRQoL instruments offer a means to monitor patients’ general disease condition, disease progression and their physical and mental well-being. Instruments including the commonly used European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-C30 and GINET21 lack, however, validation and a defined minimal clinical important difference specifically for GI-NET and P-NET patients.

Keywords: Gastroenteropancreatic neuroendocrine tumours, Health-related quality of life, Systematic literature review, Chemotherapy, Biological therapies, Peptide receptor radionuclide therapy

Core tip: Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) are slow-growing cancers that arise from diffuse endocrine cells in the gastrointestinal tract and pancreas. An increased emphasis has been placed on health-related quality of life assessment in clinical studies, using reliable and validated patient-reported outcome instruments. Long-term therapeutic options provide symptomatic relief for patients with GEP-NETs, and can slow down or stabilise disease progression, but are not curative. Thus, understanding the impact of the long-term treatment options is particularly important. The aim of this study was to perform a systematic review to assess health-related quality of life focusing on patients with inoperable metastatic GEP-NETs undergoing different treatments in order to uncover areas for future research.