John Howat

Introduction: Pancreatic neuroendocrine tumors (pNETs) are a recognized feature of tuberous sclerosis complex (TSC). The current evidence suggests that pNETs occurring in TSC may exhibit a different clinical course from sporadic cases, but their natural history remains poorly characterized. Objective: This study aimed to characterize the demographics, clinical presentation, management, and long-term outcomes of TSC-associated-pNETs and to propose possible guidelines for surveillance and management. Materials and Methods: We conducted a multicenter retrospective review of TSC-pNET patients from 6 UK TSC specialist clinics and from 3 NET referral centers, from 2008 to 2024. Data on demographics, tumor characteristics, management, and outcomes, were collected. A systematic review of the literature from 2009 to 2026 on TSC-pNETs was also performed. Results: We identified a total of 26 consecutive cases with the TSC-pNET-association in our cohort: 21 cases of pNETs from TSC specialist clinics (1.1% of the population), and 5 cases of TSC-pNETs from the NET referral centers (0.25% of the population). An additional 80 cases were identified from the published literature. We observed a wide spectrum of clinical phenotypes, with the majority being nonfunctioning pNETs (n = 24; 92%), whereas 2 patients were diagnosed with glucagonomas. Surgical intervention was the mainstay initial treatment, the indication being either functional pNETs, or large or symptomatic nonfunctioning pNETs. Conclusion: TSC-pNETs are rare and mostly nonfunctioning tumors with variable clinical behavior. Due to their uncertain malignant potential, we suggest that baseline pancreatic imaging should be incorporated into TSC surveillance, and we emphasize the need for heightened pNET surveillance and updated management recommendations.

Materially deprived communities in the UK have excess morbidity and mortality from cardiovascular disease (CVD) but are less likely to engage with formal care pathways. Community engagement and e-health may be more effective ways to promote risk-reducing lifestyle change. The “Healthy Hearts Project” website was designed for use by community health workers (CHWs) for cardiovascular risk assessment and lifestyle goal setting, or for independent use by community members. This paper describes the website’s development and evaluation. The website was developed using interactive wire frame prototypes in a user-led approach. Qualitative evaluation of the completed website’s usability and acceptability was conducted using the “Thinking Aloud” method in a purposive sample of 10 participants (one voluntary sector employee, three CHWs, two community members and four healthcare professionals). Thinking Aloud interview transcripts were thematically analysed using an inductive approach. A separate quantitative evaluation of usability and the effect of using the website on CVD knowledge and beliefs was conducted. A random sample of 134 participants, recruited using the online platform Prolific, completed the “Attitudes and Beliefs About Cardiovascular Disease” (ABCD) questionnaire before and after using the website, along with the System Usability Scale (SUS). Qualitative evaluation—Four key themes were identified: 1) Website functionality and design—participants generally found the website easy to use and understood the risk communication graphics and the feedback and goal-setting features,; 2) Inclusivity and representation—most participants considered the website inclusive of a range of users/cultures; 3) Language and comprehension–participants found the language used easy to understand but suggested reducing the amount of text; 4) Motivation and barriers to change–participants liked the personalized feedback and empowerment offered by goal-setting but commented on the need for self-motivation. Quantitative evaluation–The mean score across all domains of the ABCD questionnaire (from 2.99 to 3.11, p<0.001) and in the sub-domains relating to attitudes and beliefs around healthy eating and exercise increased after using the website. The mean(sd) score on the SUS was 77.5 (13.5). The website’s usability was generally rated well by both quantitative and qualitative measures, and measures of CVD knowledge improved after use. A number of general recommendations for the design of eHealth behaviour change tools are made based on participants’ suggestions to improve the website.