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Introduction: Maintaining adequate nutritional status can be a challenge for patients with small bowel neuroendocrine tumours (NETs). Surgical resection could result in short bowel syndrome (SBS), whilst without surgical resection there is a considerable risk of ischemia or developing an inoperable malignant bowel obstruction (IMBO). SBS or IMBO are forms of intestinal failure (IF) which might require treatment with home parenteral nutrition (HPN). Limited data exist regarding the use of HPN in patients with small bowel neuroendocrine tumours, and it is not frequently considered as a possible treatment. Methods: A systematic review was performed regarding patients with small bowel NETs and IF to report on overall survival and HPN-related complications and create awareness for this treatment. Results: Five articles regarding patients with small bowel NETs or a subgroup of patients with NETs could be identified, mainly case series with major concerns regarding bias. The studies included 60 patients (range 1–41). The overall survival time varied between 0.5 and 154 months on HPN. However, 58% of patients were alive 1 year after commencing HPN. The reported catheter-related bloodstream infection rate was 0.64–2 per 1000 catheter days. Conclusion: This systematic review demonstrates the feasibility of the use of HPN in patients with NETs and IF in expert centres with a reasonable 1-year survival rate and low complication rate. Further research is necessary to compare patients with NETs and IF with and without HPN and the effect of HPN on their quality of life. [ABSTRACT FROM AUTHOR]

Introduction: Patients with neuroendocrine neoplasms (NENs) may often develop other malignancies. This study aimed to identify the frequency at which these second malignancies occurred in England. Methods: Data were extracted from the National Cancer Registration and Analysis Service (NCRAS) on all patients diagnosed with a NEN at one of eight NEN site groups between 2012 and 2018: appendix, caecum, colon, lung, pancreas, rectum, small intestine, and stomach. WHO International Classification of Disease Edition-10 (ICD-10) codes were used to identify patients who had been diagnosed with an additional non-NEN cancer. Standardized incidence ratios (SIRs) for tumours diagnosed after the index NEN were produced for each non-NEN cancer type by sex and site. Results: A total of 20,579 patients were included in the study. The most commonly occurring non-NEN cancers after NEN diagnosis were the prostate (20%), lung (20%), and breast (15%). Statistically significant SIRs were observed for non-NEN cancer of the lung (SIR = 1.85, 95% CI: 1.55–2.22), colon (SIR = 1.78, 95% CI: 1.40–2.27), prostate (SIR = 1.56, 95% CI: 1.31–1.86), kidney (SIR = 3.53, 95% CI: 2.72–4.59), and thyroid (SIR = 6.31, 95% CI: 4.26–9.33). When stratified by sex, statistically significant SIRs remained for the lung, renal, colon, and thyroid tumours. Additionally, females had a statistically significant SIR for stomach cancer (2.65, 95% CI: 1.26–5.57) and bladder cancer (SIR = 2.61, 95% CI: 1.36–5.02). Conclusion: This study found that patients with a NEN experienced a metachronous tumour of the lung, prostate, kidney, colon, and thyroid at a higher rate than the general population of England. Surveillance and engagement in existing screening programmes are required to enable earlier diagnosis of second non-NEN tumours in these patients. [ABSTRACT FROM AUTHOR]

Simple Summary: We conducted a retrospective, population-based study comparing overall survival (OS) between males and females with neuroendocrine neoplasia (NEN). In total, 14,834 cases of NEN recorded in England's National Cancer Registry and Analysis Service (NCRAS)), were analysed. Multivariable analysis, restricted mean survival time and mediation analysis were performed. Females displayed increased survival irrespective of the stage, morphology or level of deprivation, which was statistically significant in NEN of the lung, pancreas, rectum and stomach (p < 0.001). Stage of tumour mediated improved survival in stomach, lung, and pancreatic NEN but not in rectal NEN. Females diagnosed with NEN tend to survive longer than males, and stage at presentation only accounts for part of this effect. Future research in NEN, as well as prognostication and treatment, should consider sex as an important factor. Pre-clinical studies have suggested sex hormone signalling pathways may influence tumorigenesis in neuroendocrine neoplasia (NEN). We conducted a retrospective, population-based study to compare overall survival (OS) between males and females with NEN. A total of 14,834 cases of NEN diagnosed between 2012 and 2018, recorded in England's National Cancer Registry and Analysis Service (NCRAS), were analysed. The primary outcome was OS with 5 years maximum follow-up. Multivariable analysis, restricted mean survival time and mediation analysis were performed. Appendiceal, pulmonary and early-stage NEN were most commonly diagnosed in females; stomach, pancreatic, small intestinal, colonic, rectal and later-stage NEN were more often diagnosed in males. Females displayed increased survival irrespective of the stage, morphology or level of deprivation. On average, they survived 3.62 (95% CI 1.73–5.90) to 10.26 (6.6–14.45) months longer than males; this was statistically significant in NEN of the lung, pancreas, rectum and stomach (p < 0.001). The stage mediated improved survival in stomach, lung, and pancreatic NEN but not in rectal NEN. The reasons underlying these differences are not yet understood. Overall, females diagnosed with NEN tend to survive longer than males, and the stage at presentation only partially explains this. Future research, as well as prognostication and treatment, should consider sex as an important factor. [ABSTRACT FROM AUTHOR]

The subject of colorectal neuroendocrine neoplasms (NENs), subdivided into well-differentiated NENs, termed neuroendocrine tumours (NETs; grade (G) 1 and 2), and poorly differentiated NENs, termed neuroendocrine carcinomas (NECs; G3) according to the 2010 World Health Organisation (WHO) classification, has arguably not had as much attention or study as NENs occurring in other sites. Colorectal NETs and NECs are however easier to study than many others since they are usually not difficult to remove and are increasingly detected because of intensified colorectal cancer screening and surveillance programmes. Colorectal NETs and NECs show site-specific heterogeneity with variable behaviour and different therapeutic options; these various aspects provide unique challenges. Because of bowel cancer screening programmes, colorectal NENs, like conventional adenocarcinomas, may be diagnosed at a stage that is associated with improved survival. In this article we intend to describe and define areas of unmet needs relating to the epidemiology, classification, pathology, diagnosis and therapy of colorectal NETs (including NETs G3), colorectal NECs, and finally, mixed adeno-neuroendocrine carcinomas (MANECs) by reviewing and discussing the relevant literature. [ABSTRACT FROM AUTHOR]

The gastrointestinal tract and pancreas are common primary sites for neuroendocrine tumours (NETs). Patients often report a long duration of non-specific symptoms in the year prior to diagnosis. The aims of this study were, firstly, to establish pre-diagnosis patterns of symptoms, and secondly, to determine the time from onset of symptoms to NET diagnosis and understand the interaction with primary and secondary healthcare providers. A survey was designed on a web-based survey platform with the focus on patient symptoms prior to diagnosis and a screen for functional diarrhoea (Rome III criteria [C4]). A total of 303 responses were received. The median duration from the time of first symptoms to diagnosis was 36 months for small bowel NETs and 24 months for pancreatic NETs. Common first symptoms were pain (36%), flushing (24%), and diarrhoea (24%); 29% of small bowel NET respondents were given an initial diagnosis of irritable bowel syndrome. Dyspepsia was the second most common initial incorrect diagnosis. Respondents saw their GP 5 times over a median 18-month period for their symptoms; 31% of patients were diagnosed following unplanned emergency admission. In conclusion, this survey demonstrates a median time to diagnosis of 36 months for patients with small bowel NETs. Incorrect initial diagnosis appears to be very common, with a high number of attendances in primary and secondary care prior to a correct diagnosis being made. An earlier diagnosis may improve patients’ quality of life and possible survival. [ABSTRACT FROM AUTHOR]

Purpose: Pancreatic neuroendocrine tumours (pNETs) are rare neoplasms, in that they may only present symptoms of the hormone secreted, without any generic cancer issues. It is thus important to measure quality of life (QoL) in these patients by evaluating issues relevant and important to them, as opposed to general cancer issues. This paper systematically reviews papers addressing the symptoms and QoL implications of pNETs, and evaluates each subtype separately, with the aim to create a list of QoL issues relevant to these patient groups. Methods: Medline, EMBASE, CINAHL, Psyclnfo, Web of Science, Scopus, OpenGrey, and the Cochrane Library were searched for publications (1990-2016) reporting symptoms and QoL issues in pNETs. Results: Following screening of 2,797 papers, 69 articles were eligible for data extraction. From these papers, 84 different symptoms or QoL issues were extracted: 21 for gastrinoma, 18 for glucagonoma, 50 for insulinoma, 10 for VIPoma and 15 for nonfunctioning pNETs. No issues were reported for somatostatinoma, PPoma or ACTHoma. The most frequently reported symptoms vary by subtype. Conclusions: This review emphasises the need to develop a QoL measure for pNETs with specific items relevant to the different subtypes, due to the distinct symptoms reported. Following from this review, patient and healthcare professional interviews will be conducted in large cohorts across many different countries to collect more data on QoL issues specific to pNETs. This data will all be collated with the aim to create a QoL measure for pNETs. [ABSTRACT FROM AUTHOR]