Insulinomatosis: una causa muy rara de tumor neuroendocrino pancreático

  • H. Jaramillo Chacón Universidad de los Andes
  • D. González Devia Universidad de los Andes
  • R. P. López Panqueva Universidad de los Andes
  • D. Cañon Solano Universidad de los Andes
  • D. Aguirre Matallana Hospital Universitario Fundación Santa Fe de Bogotá
  • A. M. Rey Rubiano Hospital Universitario Fundación Santa Fe de Bogotá
  • J. M. Segovia Gómez Hospital Universitario Fundación Santa Fe de Bogotá
  • R. F. Dussan Flórez Hospital Universitario Fundación Santa Fe de Bogotá

Resumen

Los tumores neuroendocrinos pancreáticos representan del 2-10 % de todos los tumores del páncreas y aproximadamente el 7 % de todos los tumores neuroendocrinos. Estos se clasifican como funcionales o no funcionales según la presencia o ausencia de síndromes clínicos asociados con la hipersecreción hormonal. Los insulinomas son los tumores neuroendocrinos pancreáticos funcionales más frecuentes (45 % de los casos) y la causa más frecuente de hipoglucemia hiperinsulinémica endógena persistente en adultos. Además, el 10 % de los tumores neuroendocrinos pancreáticos se asocian con neoplasia endocrina múltiple tipo 1. La insulinomatosis es una entidad clínica distinta en la que existen múltiples insulinomas.
Objetivos: exponer los casos reportados hasta el momento de insulinomatosis y describir las causas genéticas, las características clínicas, el tratamiento, y el pronóstico de la insulinomatosis.
Métodos: se realizó una búsqueda sobre insulinomatosis y los factores que controlan la proliferación de las células β en las bases de datos PubMed, Medline y Google Scholar hasta Julio 2020.
Resultados: 108 casos con insulinomatosis se han reportado hasta la fecha, siendo recurrente y rara vez malignos. Múltiples protooncogenes y supresores de tumores controlan de forma local y sistémica el crecimiento de las células β; sin embargo, solo la mutación de MafA en p.Ser64Phe ha sido asociada.
Conclusión: la insulinomatosis se caracteriza por la aparición sincrónica y metacrónica de insulinomas. Tiene un fenotipo histológico, clínico y genético diferente a los tumores neuroendocrinos pancreáticos; la mutación MEN-1 es negativa; puede ser esporádica o hereditaria; y MafA podría ser una mutación conductora.

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Biografía del autor

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MD, Pregrado de medicina, Universidad de los Andes. Bogotá D. C., Colombia.

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MD, MsC, medicina interna, endocrinología. Hospital Universitario Fundación Santa Fe de Bogotá, Universidad de los Andes.

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MD, patología quirúrgica. Hospital Universitario Fundación Santa Fe de Bogotá, Universidad de los Andes.

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MD, patología quirúrgica. Hospital Universitario Fundación Santa Fe de Bogotá, Universidad de los Andes.

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MD, radiología, Hospital Universitario Fundación Santa Fe de Bogotá.

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MD, medicina interna, gastroenterología. Hospital Universitario Fundación Santa Fe de Bogotá, Universidad de los Andes.

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MD, medicina interna, hematooncología. Hospital Universitario Fundación Santa Fe de Bogotá, Universidad de los Andes.

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MD, radioterapia. Hospital Universitario Fundación Santa Fe de Bogotá, Universidad de los Andes.

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Publicado
2020-09-17
##submission.howToCite##
JARAMILLO CHACÓN, H. et al. Insulinomatosis: una causa muy rara de tumor neuroendocrino pancreático. Revista Colombiana de Endocrinología, Diabetes & Metabolismo, [S.l.], v. 7, n. 2, p. 76-85, sep. 2020. ISSN 2389-9786. Disponible en: <http://revistaendocrino.org/index.php/rcedm/article/view/607>. Fecha de acceso: 20 oct. 2020
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