Insulinomatosis: una causa muy rara de tumor neuroendocrino pancreático
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Palabras clave

hipoglucemia
insulinoma
nesidioblastosis
insulinomatosis
tumor neuroendocrino

Cómo citar

Jaramillo Chacón, H., González Devia, D., López Panqueva, R. P., Cañon Solano, D., Aguirre Matallana, D., Rey Rubiano, A. M., Segovia Gómez, J. M., & Dussan Flórez, R. F. (2020). Insulinomatosis: una causa muy rara de tumor neuroendocrino pancreático. Revista Colombiana De Endocrinología, Diabetes &Amp; Metabolismo, 7(2), 76–85. https://doi.org/10.53853/encr.7.2.607

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 ? 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 ?; 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.

https://doi.org/10.53853/encr.7.2.607
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Citas

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