Distinct Clinical Characteristics in Young-Onset Pancreatic Neuroendocrine Tumor

Area of Science:

• Oncology
• Endocrinology
• Genetics

Background:

• Pancreatic neuroendocrine tumors (PNET) can occur at various ages.
• Young-onset PNET (YOPNET) and typical-onset PNET (TOPNET) may have different characteristics and outcomes.

Purpose of the Study:

• To compare socioeconomic factors, molecular characteristics, and survival between YOPNET and TOPNET patients.
• To identify prognostic factors for survival in PNET based on age of onset.

Main Methods:

• Retrospective analysis of 6259 PNET patients from the National Cancer Database (2004-2016).
• Comparison of YOPNET (<50 years) and TOPNET (≥50 years) using Kaplan-Meier and Cox regression.
• Propensity score matching and genomic dataset analysis for mutation frequencies.

Main Results:

• YOPNET patients were younger, more diverse ethnically, and had higher tumor stage/size but better overall survival (OS).
• YOPNET remained an independent prognostic factor for improved OS post-matching.
• YOPNET showed a significantly lower rate of MEN-1 mutations compared to TOPNET.

Conclusions:

• YOPNET is a distinct clinical entity with improved survival despite aggressive features.
• Socioeconomic and molecular differences, including lower MEN-1 mutation rates, characterize YOPNET.