Gathering data is vital, and we get involved in a number of research projects both Nationally and Internationally, ensuring that the patient perspective is heard. Below you will see a snapshot of some of our work. Thank you to each and every one of you who completes a questionnaire or responds to a survey. It is SO important to be able to share your views – to bring your voice to a platform where it can be listened to and acted on. If you think you can help or you are willing to take part in any of our research projects then do let us know.
We have also listed publications from around the globe.
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Sex differences and survival of neuroendocrine neoplasia in England 2012-2018
Authors: Benjamin Easton White, Kandiah Chandrakumaran, Kwok Wong, Brian Rous
Incidence and Survival Outcomes in Patients with Lung Neuroendocrine Neoplasms in the United States
Published: 2021
Abstract Background: The incidence and prevalence of neuroendocrine neoplasms (NENs) are rapidly rising. Epidemiologic trends have been reported for common NENs, but specific data for lung NENs are lacking. Methods: We conducted a retrospective analysis utilizing the Surveillance, Epidemiology, and End Results (SEER) database. Associated population data were utilized to report the annual age-adjusted incidence and overall survival (OS) trends. Trends for large-cell neuroendocrine carcinoma (LCNEC) and atypical carcinoid (AC) were reported from 2000–2015, while those for typical carcinoid (TC) and small cell lung cancer (SCLC) were reported from 1988–2015. Results: We examined a total of 124,969 lung NENs [103,890—SCLC; 3303—LCNEC; 8146—TC; 656—AC; 8974—Other]. The age-adjusted incidence rate revealed a decline in SCLC from 8.6 in 1988 to 5.3 in 2015 per 100,000; while other NENs showed an increase: TC increased from 0.57 in 1988 to 0.77 in 2015, AC increased from 0.17 in 2001 to 0.22 in 2015, and LCNEC increased from 0.16 in 2000 to 0.41 in 2015. The 5-year OS rate among SCLC, LCNEC, AC, and TC patients was 5%, 17%, 64%, and 84%, respectively. On multivariable analyses, OS and disease-specific survival (DSS) varied significantly by stage, sex, histological type, insurance type, marital status, and race, with a better survival noted in earlier stages, females, married, insured, Hispanic and other races, and urban population. Similarly, TC and AC had better survival compared to SCLC and LCNEC. Conclusion: The incidence of lung NENs is rising, possibly in part because of advanced radiological techniques. However, the incidence of SCLCs is waning, likely because of declining smoking habits. Such population-based studies are essential for resource allocation and to prioritize future research directions.
Authors: Shrunjal Shah,Rohit Gosain, Adrienne Groman, Rahul Gosain, Arvind Dasari, Thorvardur R. Halfdanarson, and Sarbajit Mukherjee
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NCUK’s: Patient Perspectives, from Diagnosis Through Treatments & Beyond
Published: 2021
Abstract The purpose of this review is to reflect on the current patient experience of Neuroendocrine Cancer (NC) from the prediagnosis stage to identify recommendations that will provide quality care through patient centred decision-making. A review of the latest patient led research around the continued delays in a correct diagnosis, the barriers causing that delay or misdiagnosis, highlights clearly both on a global scale, and UK specific that the faces of the patients may change, but the facts remain the same. In the UK, there is a low suspicion of NC among both the patient and healthcare community at initial presentation of symptoms, with less than 4% of primary care physicians suspecting any form of cancer at all, and if patients were referred on, in less than 17% of secondary care physicians was cancer suspected (Basuroy et al., 2018) . Globally, 44% of 2359 patients (1043) were initially misdiagnosed [4]. Challenges continue once diagnosed around access, the prospect of multiple treatments, side effects and longer-term consequences, lifelong tests, hospital appointments and the undeniable reality of living with persistent and pervasive uncertainty. With the prospect of living with an incurable cancer, emotional and quality of life considerations should be of equal importance; “I am not afraid of dying, but I am afraid of not living”. There is also a need to see a truly collaborative approach, across all stakeholders that incorporates and prioritises patient identified care needs and priorities. Patients as partners in care is an absolute requirement for improvements in both experience and outcomes.
Authors: Catherine Bouvier and Nicola Jervis
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The Role of Patient Support Groups in Neuroendocrine Neoplasms
Published: 2021
Abstract Purpose of Review The purpose of this review is to establish the role patient support groups play in NENs. Recent Findings Published data on the role and work done by these groups is extremely sparse, so the review references publications in the wider cancer advocacy context. For the purposes of the review, a survey was carried out among the members of a global umbrella organization to ascertain the level of activities undertaken in support of the NEN patient community. Summary The concept of “support groups” has changed significantly, as these groups have evolved from patient peer-to-peer support provision to a strategic focus on improving awareness and education among all stakeholders, generating patient evidence to influence policies for access to optimal diagnostics, treatment, and care, and setting the research agenda. Today, NEN patient organizations have an instrumental role of catalysts of change across the healthcare spectrum—especially relevant in a setting of less common and not well-understood diseases, where clear pathways and guidelines are still a challenge.
Authors: Teodora Kolarova & Catherine Bouvier
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Evaluating cost-effectiveness in the management of neuroendocrine neoplasms
Abstract The rapid evolution of novel, costly therapies for neuroendocrine neoplasia (NEN) warrants formal high-quality cost-effectiveness evaluation. Costs of individual investigations and therapies are high; and examples are presented. We aimed to review the last ten years of standalone health economic evaluations in NEN. Comparing to published standards, EMBASE, Cochrane library, Database of Abstracts of Reviews of Effects (DARE), NHS Economic Evaluation Database and the Health Technology Assessment (HTA) Database were searched for health economic evaluations (HEEs) in NEN published between 2010 and October 2019. Of 12 economic evaluations, 11 considered exclusively pharmacological treatment (3 studies of SSAs, 7 studies of sunitinib, everolimus and/or 177Lu-DOTATATE and 1 study of telotristat ethyl) and 1 compared surgery with intraarterial therapy. 7 studies of pharmacological treatment had placebo or best supportive care as the only comparator. There remains a paucity of economic evaluations in NEN with the majority industry funded. Most HEEs reviewed did not meet published health economic criteria used to assess quality. Lack of cost data collected from patient populations remains a significant factor in HEEs where clinical expert opinion is still often substituted. Further research utilizing high-quality effectiveness data and rigorous applied health economic analysis is needed.
Authors: B. E. White, R. Mujica-Mota, T. Snowsill, E. M. Gamper, R. Srirajaskanthan & J. K. Ramage
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Health-related quality of life in neuroendocrine neoplasia: a critical review
Published: 2020
Abstract Incidence of neuroendocrine neoplasia (NEN) is increasing, as is use of health-related quality of life (HRQoL) measurement in clinical trials. Following development of validated questionnaires, HRQoL is widely used to assess outcomes. This review is intended for healthcare professionals and is based on a selection of data published in the last decade. HRQoL is on par with other clinical endpoints such as performance status. Assessments in clinical trials have been particularly useful for monitoring the symptom burden of NEN, for the effects of treatments on patients’ lives, and have provided new data allied to the usual clinical endpoints. QoL expressed as quality-adjusted life years (QALYs) have become the most important primary outcome to establish cost-effectiveness in health economic evaluation. From looking at clinical trials over the last 10 years, we see that the quality of HRQoL evidence reported in published studies has improved and, in general, recent studies are likely to be more methodologically robust. Assessment of HRQoL in clinical trials is likely to become a standard part of clinical practice in NEN, as in other cancers. However, clear methods for calculating the clinical meaningfulness of changes in scores are needed. Other limitations of HRQoL measurement include lack of specificity to certain symptom sets and ease of completion and administration. An international group taking a lead on developing HRQoL research specifically in NEN patients is needed to address limitations of the evidence base. In order for greater weight to be placed on HRQoL data, agreement on optimal, validated scoring systems is needed.
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NCUK’s: COVID & My Care Report
Published: 2020
There is a real concern regarding the huge impact the Covid-19 Pandemic has had, and will have on healthcare for patients with cancer. To understand the impact this has had on the Neuroendocrine Cancer Community, a group often thought of as chronically rather than acutely affected, we undertook a survey. Utilising Survey Monkey, Neuroendocrine Cancer UK’s social media platforms, patient database and NCUK website, we sought to obtain a snapshot of the effect this pandemic has had on patients’ healthcare access and psychosocial needs. We obtained responses from 361 participants from a fairly evenly geographical distribution across the whole of the United Kingdom. The majority of individuals completing the survey were linked to a Specialist Centre – the slightly higher proportion in the South East maybe reflecting the concentration of Centres within that region. Most of the respondents had a diagnosis of GEP or Lung based Neuroendocrine Cancer.
Author: Nikie Jervis
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NCUK’s: Barriers to Diagnosis Report
Published: 2020
The purpose of the NCUK Barriers Survey was to consolidate and add to previous national and global Neuroendocrine Cancer (NC)* patient experience surveys and reports, to gain further insights into the patient pathway for those diagnosed with NC. From initial symptom, through seeking advice to diagnostics and treatments – does current experience of Neuroendocrine Cancer care confirm the influential factors identified in previous cancer care reports?
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Unmet needs in the international neuroendocrine tumor (NET) community: Assessment of major gaps from the perspective of patients, patient advocates and NET health care professionals
Published: 2019
Abstract Due to the increasing incidence and prevalence of neuroendocrine tumors (NETs), there is a need to assess any gaps in awareness and care. A survey was undertaken in 2017 to identify perceived unmet needs from the perspectives of patients/families, patient advocates and health care professionals (HCPs). The survey consisted of 33–37 questions (depending on type of respondent) across four areas: information, care, treatments and research. In total, 443 participants from 26 countries responded: 338 patients/families, 35 advocates and 70 HCPs. Perceived unmet needs regarding provision of information at diagnosis differed between groups. While 59% of HCPs believed they provided sufficient information, informational needs were mostly/fully met for only 30% of patients and 18% of advocates. Additionally, 91% of patients and 97% of advocates felt that patients had to search for information themselves. Availability of Gallium‐68‐Dotatate PET/CT scan was limited for the majority of patients (patients: 73%; advocates: 85%; HCP: 86%), as was access to treatments, particularly peptide receptor radionuclide therapy (patients: 42%; advocates: 95%; HCPs: 77%). All groups felt that standards of care, including psychological needs and diagnosis of mental health, were not fully met. Although about two‐thirds of patients were managed by a multidisciplinary team, 14% of patients reportedly did not have enough contact. All groups supported more patient involvement in research; patients and advocates prioritized improvement in diagnosis and HCPs focused on clinical trials. This survey revealed significant unmet needs but differing perceptions regarding these among the groups. There is a need for investigation and collaboration to improve standards of care for NET patients.
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Impact of neuroendocrine morphology on cancer outcomes and stage at diagnosis: a UK nationwide cohort study 2013–2015
Published: 2019
BACKGROUND: The diagnosis of neuroendocrine neoplasms (NENs) is often delayed. This first UK population-based epidemiological study of NENs compares outcomes with non-NENs to identify any inequalities. METHODS: Age-standardised incidence rate (ASR), 1-year overall survival, hazard ratios and standardised mortality rates (SMRs) were calculated for all malignant NENs diagnosed 2013–2015 from UK national Public Health records. Comparison with non-NENs assessed 1-year overall survival (1YS) and association between diagnosis at stage IV and morphology. RESULTS: A total of 15,222 NENs were identified, with an ASR (2013–2015 combined) of 8.6 per 100,000 (95% CI 8.5–8.7); 4.6 per 100 000 (95% CI, 4.5–4.7) for gastro-entero-pancreatic (GEP) NENs. The 1YS was 75% (95% CI, 73.9–75.4) varying significantly by sex. Site and morphology were prognostic. NENs (predominantly small cell carcinomas) in the oesophagus, bladder, prostate, and female reproductive organs had a poorer outcome and were three times more likely to be diagnosed at stage IV than non-NENs. CONCLUSION: Advanced stage at diagnosis with significantly poorer outcomes of some NENs compared with non-NENs at the same anatomical site, highlight the need for improved access to specialist services and targeted service improvement.
Authors: Tracey S. E. Genus, Catherine Bouvier, Kwok F. Wong, Rajaventhan Srirajaskanthan, Brian A. Rous, Denis C. Talbot, Juan W. Valle, Mohid Khan, Neil Pearce, Mona Elshafie, Nicholas S. Reed, Eileen Morgan, Andrew Deas, Ceri White,, Dyfed Huws and John Ramage
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Presenting Symptoms and Delay in Diagnosis of Gastrointestinal and Pancreatic Neuroendocrine Tumours
Published: 2018
Abstract 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. Authors: Ron Basuroy, Cathy Bouvier, John K. Ramage, Maia Sissons, Alexandra Kent, Raj Srirajaskanthan
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Prevalence of self-reported side effects in neuroendocrine tumour patients prescribed somatostatin analogues
Published: British Journal of Nursing, 2018
Abstract Somatostatin analogues (SSA) are a common treatment for some forms of neuroendocrine tumours (NETs). Patients report a variety of side effects after starting these drugs, so in most cases they require a lot of nutritional input. The authors used an online survey to invite responses from patients worldwide to determine the extent of reported side effects. Patients were asked which SSA they were taking and how they rated the severity of their side effects. They were provided with a list of 11 options to choose from, but not given any guidance or a definition of terms. The most commonly reported side effect was constipation (85%), with 8.6% of these respondents rating its severity as 10/10. The survey found that many self‑reported side effects from the use of SSAs were experienced more frequently than previous clinical studies or the patient information leaflets reported. As this was an online survey, so the findings are limited in that first, this was a self-selected sample of patients and second, patients were able to respond more than once.
Authors: Tara Whyand, Catherine Bouvier and Philippa Davies
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Delays and routes to diagnosis of neuroendocrine tumours
Published: 2018
Abstract Background – Neuroendocrine tumours are uncommon tumours; there is often a long period between the onset of symptoms and diagnosis. This study aims to address the symptoms prior to diagnosis of people with known neuroendocrine tumours and also the involvement of healthcare providers prior to the diagnosis.
Methods – A web based survey was designed to cover two broad areas of patient symptoms and healthcare interactions prior to diagnosis. This was tested and adapted by patient and clinician input prior to distribution via Survey Monkey.
Results – The results demonstrated a median time from first symptom to diagnosis of 53.8 months. The most frequent initial symptoms were of pain, change in bowel habit and fatigue. 31% of respondents noted weight loss prior to diagnosis. 80% of respondents visited their GP regarding the symptoms a median of 11 times. 58% of respondents were referred to secondary care where they were seen a median 3 times. 30% presented acutely to A&E and this led to their diagnosis.
Conclusion – In conclusion, there is a long time from onset of symptoms to diagnosis in all types of NETs. This is despite many respondents having alarm symptoms at diagnosis. Further education and awareness regarding malignancy may help with earlier diagnosis.
Authors: Ron Basuroy, Catherine Bouvier, John Keith Ramage, Maia Sissons & Raj Srirajaskanthan
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NCUK’s: Widening the NET
Published: 2017
This report is focused on improving outcomes for patients with Neuroendocrine Cancer in Scotland it outlines ‘ambitions’ and ‘actions’ to ensure that progress continues to be made in Scottish NET care.
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NCUK’s: NCUK Where is Chapter 10?
Published: 2015
One-Size Fits No-One: How can we improve the impact and outcomes for patients who follow a less common pathway?
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NCUK’s: Patient Experience Survey
Published: 2015
Each year in the UK, around 5 in every 100,000 people are diagnosed with neuroendocrine tumours (NETs). Neuroendocrine tumours are uncommon and because of their nature, gathering data to look at patients’ experience of care and treatment is very challenging. In 2014, the NET Patient Foundation commissioned Quality Health to undertake a survey of people who were undergoing treatment for neuroendocrine cancer. The survey aimed to gain valuable data on the diagnosis, treatment and care of, and information and support services for neuroendocrine cancer patients in order to improve the quality of health services for future patients. Because of the difficulty in identifying NET patients, samples were drawn by working directly with 7 NHS Trusts in England providing specialist NET treatment. The 7 NHS Trusts which took part in the survey were: • Imperial College Healthcare NHS Trust • King’s College Hospital NHS Foundation Trust • Oxford University Hospitals NHS Trust • Royal Free London NHS Foundation Trust • The Christie NHS Foundation Trust • The Royal Liverpool and Broadgreen University Hospitals NHS Trust • University Hospitals Coventry and Warwickshire NHS Trust.
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