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Is Routine Screening of Young Asymptomatic MEN1 Patients Necessary?

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Abstract

Background

Recent clinical practice guidelines recommend that routine screening of MEN1 mutation carriers should start at the age of 5 years. The occurrence of clinically relevant MEN1 organ manifestations in children (≤18 years) was evaluated.

Methods

Two prospective collected databases of MEN1 patients (n = 166) who underwent annual screening were retrospectively analyzed for organ manifestations in MEN1 patients ≤18 years. The follow-up was based on the most recent screening examination until December 2015.

Results

Twenty [11 females, 9 males, (12%)] of 166 MEN1 patients were diagnosed with at least one organ manifestation at age ≤18 years. The most frequent manifestation was mild asymptomatic pHPT (n = 9, 45%, age range 8–18 years). Eight (40%) young patients had pNENs (three non-functioning pNENs, five insulinomas, age range 9–18 years). All five insulinomas were diagnosed based on hypoglycemic symptoms. The other organ manifestations were asymptomatic pituitary adenomas in six patients (30%, age range 15–18 years) and a bronchial carcinoid in one 15-year-old patient. Only six (30%) patients ≤18 years had clinically relevant organ manifestations.

Conclusion

Symptomatic or severe manifestations in MEN1 patients rarely occur below the age of 16 years. With regard to psychological burden and cost-effectiveness, routine screening of asymptomatic MEN1 patients should be postponed at least until the age of 16 years.

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Acknowledgements

We are grateful to all patients that participated in the study.

Funding

This research did not receive any specific grant from any funding agency in the public, commercial or nonprofit sector.

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Authors and Affiliations

  1. Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Baldingerstrasse, 35041, Marburg, Germany

    Jerena Manoharan, Caroline L. Lopez, Max B. Albers, Carmen Bollmann, Volker Fendrich, Emily P. Slater & Detlef K. Bartsch

  2. Practice and Molecular Laboratory, Brueckenstrasse 21, 69120, Heidelberg, Germany

    Friedhelm Raue

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  1. Jerena Manoharan
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  2. Friedhelm Raue
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  3. Caroline L. Lopez
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Correspondence to Jerena Manoharan.

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Manoharan, J., Raue, F., Lopez, C.L. et al. Is Routine Screening of Young Asymptomatic MEN1 Patients Necessary?. World J Surg 41, 2026–2032 (2017). https://doi.org/10.1007/s00268-017-3992-9

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  • DOI: https://doi.org/10.1007/s00268-017-3992-9

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