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Uncommon tumors in multiple endocrine neoplasia (MEN) type 1: Do they have a relationship with the prognosis of these patients?

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Abstract

Introduction

The prognosis of MEN 1 patients is not only determined by pancreatic disease; it is also related to other uncommon tumors. The objective of this study is to analyze the tumors associated with MEN 1 outside the classic triad and to investigate their relationship with mortality.

Materials and methods

One hundred and five MEN 1 patients were studied in a tertiary referral hospital (1980–2019).

Results

With a follow-up of 11 ± 4 years, seven patients died (8%), four as a consequence MEN syndrome. Thirty-three percent had adrenal gland tumors. One patient died of adrenal cancer. Eight percent presented with a neuroendocrine thoracic neoplasm, and one patient died. Another patient died due to cutaneous T-cell lymphoma. A further patient died because of a gastrinoma with liver metastasis.

Conclusions

To conclude, 75% of MEN-related deaths were the result of an uncommon pathology, and we, therefore, recommend that these tumors should be taken into account in the screening and follow-up of these patients.

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Authors and Affiliations

  1. General Surgery Service, Endocrine Surgery Unit, Instituto Murciano de Investigación Biosanitaria (IMIB), Virgen de La Arrixaca University Hospital, Crta./Madrid-Cartagena, S/N, 30120, El Palmar, Murcia, Spain

    B. Febrero, J. J. Ruiz-Manzanera, I. Ros, A. Ríos & J. M. Rodríguez

  2. Endocrinology Service, Virgen de la Arrixaca University Hospital, 30120, Murcia, Spain

    P. Segura & A. M. Hernández

  3. Maxillofacial Surgery Service, Virgen de la Arrixaca University Hospital, 30120, Murcia, Spain

    E. Teruel

Authors
  1. B. Febrero
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  2. P. Segura
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  3. J. J. Ruiz-Manzanera
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  4. E. Teruel
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  5. I. Ros
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  6. A. Ríos
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  7. A. M. Hernández
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  8. J. M. Rodríguez
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Correspondence to B. Febrero.

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Febrero, B., Segura, P., Ruiz-Manzanera, J.J. et al. Uncommon tumors in multiple endocrine neoplasia (MEN) type 1: Do they have a relationship with the prognosis of these patients?. J Endocrinol Invest 44, 1327–1330 (2021). https://doi.org/10.1007/s40618-020-01414-2

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  • DOI: https://doi.org/10.1007/s40618-020-01414-2

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