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Diagnosing Nonfunctional Pancreatic NETs in MEN1: The Evidence Base
Mark J C van Treijen1, Dirk-Jan van Beek2, Rachel S van Leeuwaarde1
1Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, Netherlands.
Journal of the Endocrine Society
|September 12, 2018
Summary
Nonfunctional pancreatic neuroendocrine tumors (NF-pNETs) in multiple endocrine neoplasia type 1 (MEN1) require updated diagnostic strategies. Biomarkers are unreliable; endoscopic ultrasound (EUS) and MRI are recommended for NF-pNET detection.
Area of Science:
- Endocrinology
- Oncology
- Diagnostic Imaging
Background:
- Nonfunctional pancreatic neuroendocrine tumors (NF-pNETs) are common in multiple endocrine neoplasia type 1 (MEN1) and a significant cause of mortality.
- Current diagnostic approaches for NF-pNETs in MEN1 lack consensus, necessitating an evidence-based review.
Purpose of the Study:
- To systematically review the diagnostic accuracy of biomarkers and imaging modalities for NF-pNETs in MEN1.
- To assess age-related penetrance and tumor growth for optimizing screening protocols.
Main Methods:
- Systematic literature search of three databases for diagnostic accuracy studies (n=27) and screening-related data (n=12).
- Critical appraisal using modified Quality Assessment of Diagnostic Accuracy Studies (QUADAS).
Main Results:
- Biomarker diagnostic accuracy for NF-pNETs is insufficient; they should not be used.
- Endoscopic ultrasound (EUS) demonstrates the highest sensitivity for NF-pNET detection, with a combined EUS and MRI strategy being most effective.
- Gallium 68 octreotate-DOTA PET-CT can aid in metastasis detection. NF-pNET growth rates are generally low, with two observed phenotypes.
Conclusions:
- Current evidence suggests abandoning biomarkers for NF-pNET diagnosis in MEN1.
- EUS and MRI are the preferred imaging modalities for NF-pNET detection. Further research is needed to establish optimal screening initiation age.
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