Abstract
CONTEXT: Nonfunctioning pancreatic neuroendocrine tumors (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: The aim of this work is to assess the age of occurrence of clinically relevant NF-pNETs in young MEN1 patients.
METHODS: Pancreatic imaging data of MEN1 patients were retrieved from the DutchMEN Study Group database. 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. 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 years, 2 of whom subsequently developed lymph node metastases. No differences in clinically relevant NF-pNET-free survival were found for sex, time frame, 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 tumor 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 to 14 years is justified. The psychological and medical burden of screening at a young age should be considered.
Original language | English |
---|---|
Pages (from-to) | 3515-3525 |
Number of pages | 11 |
Journal | Journal of Clinical Endocrinology & Metabolism |
Volume | 106 |
Issue number | 12 |
DOIs | |
Publication status | Published - Dec 2021 |
Keywords
- Adolescent
- Adult
- Age of Onset
- Aged
- Child
- Child, Preschool
- Databases, Factual
- Diagnostic Imaging
- Early Detection of Cancer/methods
- Female
- Follow-Up Studies
- Humans
- Male
- Middle Aged
- Multiple Endocrine Neoplasia Type 1/physiopathology
- Netherlands/epidemiology
- Neuroendocrine Tumors/diagnosis
- Pancreatic Neoplasms/diagnosis
- Prognosis
- Retrospective Studies
- Survival Rate
- Tumor Burden
- Young Adult
- ENDOCRINE NEOPLASIA TYPE-1
- INTERVAL-CENSORED-DATA
- surveillance
- multiple endocrine neoplasia type 1
- age-related penetrance
- pancreatic NET