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MEN1 Surveillance Guidelines: Time to (Re)Think?

Paul J Newey1, John Newell-Price2

  • 1Division of Molecular and Clinical Medicine, Ninewells Hospital & Medical School, University of Dundee, Dundee DD2 1UB, Scotland, UK.

Journal of the Endocrine Society
|January 26, 2022
PubMed
Summary

Current guidelines for multiple endocrine neoplasia type 1 (MEN1) recommend intensive surveillance. However, evidence for screening benefits and harms is limited, necessitating a patient-centered approach.

Keywords:
MEN1bronchial neuroendocrine tumorgenetic testingmultiple endocrine neoplasia type 1pancreatic neuroendocrine tumorscreeningsurveillancethymic

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Area of Science:

  • Endocrinology
  • Oncology
  • Genetics

Background:

  • Multiple Endocrine Neoplasia type 1 (MEN1) is a genetic disorder causing tumors in various endocrine and nonendocrine tissues.
  • MEN1 leads to significant premature morbidity and mortality, underscoring the need for effective management strategies.

Purpose of the Study:

  • To evaluate the ongoing utility of current surveillance options for MEN1 patients.
  • To highlight uncertainties and challenges in existing MEN1 screening guidelines, particularly for presymptomatic tumors.

Main Methods:

  • Review of current clinical practice guidelines for MEN1 surveillance.
  • Analysis of the evidence base supporting various screening modalities.
  • Consideration of potential iatrogenic harms and patient-centered care.

Main Results:

  • Current MEN1 guidelines recommend intensive clinical, biochemical, and radiological surveillance from early childhood.
  • The evidence supporting the benefits of several MEN1 screening aspects is not robust.
  • Potential iatrogenic harms and the psychological/economic burdens of intensive screening are not well-studied.

Conclusions:

  • While surveillance is crucial for MEN1, current guidelines face limitations and uncertainties.
  • A patient-centered screening approach is advocated, acknowledging current evidence gaps.
  • Further research is needed to refine MEN1 surveillance strategies and patient management.