Context
Non-functioning pancreatic neuroendocrine tumours (NF-pNETs) are highly prevalent and constitute an important cause of mortality in patients with multiple endocrine neoplasia type 1 (MEN1). Still, the optimal age to initiate screening for pNETs is under debate.
Objective
To assess the age of occurrence of clinically relevant NF-pNETs in young MEN1 patients.
Patients and setting
Pancreatic imaging data of MEN1 patients were retrieved from the DutchMEN Study Group database.
Design
Interval-censored survival methods were used to describe age-related penetrance, compare survival curves, and develop a parametric model for estimating the risk of having clinically relevant NF-pNET at various ages.
Intervention(s)
Not applicable.
Main outcome measure(s)
The primary objective was to assess age at occurrence of clinically relevant NF-pNET (size ≥20 mm or rapid growth); secondary objectives were the age at occurrence of NF-pNET of any size and pNET-associated metastasized disease.
Results
Five of 350 patients developed clinically relevant NF-pNETs before age 18, two of which subsequently developed lymph node metastases. No differences in clinically relevant NF-pNET-free survival were found for sex, timeframe, and type of MEN1 diagnosis or genotype. The estimated ages (median, 95% CI) at a 1%, 2.5% and 5% risk of having developed a clinically relevant tumour are 9.5 (6.5 – 12.7), 13.5 (10.2 – 16.9) and 17.8 years (14.3 – 21.4) respectively.
Conclusion
Analyses from this population-based cohort indicate that start of surveillance for NF-pNETs with pancreatic imaging at age 13–14 is justified. The psychological and medical burden of screening at a young age should be considered.
Targeted screening programs for individuals with an increased risk for cancer have become increasingly available. Patients with multiple endocrine neoplasia (MEN), rare genetic conditions associated with the development of tumors in the endocrine glands, undergo intensive surveillance from an early age. Quantitative research has shown that patients with MEN experience fear of disease occurrence in themselves and their family members. However, little is known about the role that intensive, lifelong screening plays in the lives of individuals. This study investigates the lived experiences of patients with MEN undergoing regular tumor screening through an interpretative phenomenological analysis of interviews with 12 patients with MEN1, MEN2A, or MEN2B syndrome. Four experiential group themes are identified: coming to the foreground/fading into the background, relating to uncertainty, experiencing control, and familial context. Screening is characterized as an ambiguous experience that brings MEN to the foreground and may both exacerbate MEN‐related uncertainty as well as provide a sense of control over the disease. The experience of undergoing screening is strongly influenced by the familial context, as participants care for and are cared for by family members and understand their disease through familial experiences. Good care according to patients with MEN includes providing family‐centered care, addressing the impact on daily functioning and the meaning of illness, support in the interpretation of physical complaints, facilitation of patient experiences of control, and careful attunement to patient needs within a good doctor–patient relationship.
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