Insulinoma y Neoplasia Endocrina Múltiple Tipo I

Estadísticamente se ha observado que un 10% de los pacientes con hiperinsulinismo tienen un síndrome MEN 1. El insulinoma es el segundo tumor funcionante de células de los islotes más común en este síndrome familiar. La presencia de un MEN I puede ser determinada por una cuidadosa historia familiar y la búsqueda de otras manifestaciones del síndrome. Los niveles precisos en el suero de calcio, prolactina y polipéptidos pancreáticos pueden ser útiles para detectar su presencia (87, 88).

El páncreas de pacientes con síndrome MEN 1, siempre tienen múltiples tumores de los islotes pancreáticos, y los estudios recientes sugieren que el duodeno también tiene múltiples tumores neuroendocrinos (87-89). Sin embargo, estudios cuidadosos con inmunoperoxidasa hacen ver que los tumores pancreáticos son insulinomas (90). Otro hecho importante es que los grandes tumores pueden ser malignos. Los pacientes con MEN I e insulinoma, generalmente tienen un tumor de las células de los islotes dominante, capaz de ser localizado por imágenes y ser el responsable de la excesiva secreción de insulina, además de la identificación de otros tumores pequeños. Si el tumor dominante está presente dentro del cuerpo o la cola del páncreas, se recomienda una pancreatectomía distal o subtotal. Si el tumor dominante está en la caheza pancreática, se de he intentar la enucleación. Si esto no es posible y el tumor es grande y está en la cercanía del canal principal, existe indicación para la pancreatoduodenectomía.

No obstante el hecho de que estos pacientes tienen múltiples tumores de los islotes y que algunos pueden ser malignos, ellos permanecen libres de hipoglicemia después de la resección quirúrgica. Se requiere un cuidadoso seguimiento debido a que puede desarrollarse un hiperinsulinismo recurrente ya sea por la recurrencia de otro insulinoma o por desarrollo de enfermedad metastásica del tumor original. Las resecciones quirúrgicas repetidas están indicadas para el hiperinsulinismo recurrente en pacientes con MEN 1, SI el procedimiento conlleva aceptable morbimortalidad.

La presencia de otras endocrinopatías como el hiperparatiroidismo primario en pacientes con MEN J, puede afectar los síntomas relacionados con los tumores de los islotes; por lo tanto, se recomienda eliminar por cirugía primero el hiperparatiroidismo primario, para mejorar el control del tumor de células insulares.

Abstract

With the presentation of 8 cases of benign insulinomas, a review of current concepts on gastroenteropancreatic neuroendocrine tumor, is made. With relation to insulinomas, alfention is ml/ed upon fasting hypoglicemia. emphasiúng that this one is more severe than other causes of low blood sugar, always implicating. in adults. a high degree (of hyperinsulinism. Complementan” laboraton” studies are also of great importanCe for the recognition of the inappropriate secretion of insulin and other pancreatic peptides, through the concomitant elevation of plasmatic glucose and insulin; thus establishing the circulating insulin/ilucose ratio.

Emphasis is made on the use of se!ective arteriography and intra-operatory ultrasonographv for the diagnosis of insulin secreting tumors. The first method offers the greatest sensibility for localization of the tumor and the lalfer is (j{ great use when preoperative imageneological studies are inconc/usive. speciallv with ven” smal/ tumors.Finally. the complete enuc/eation o{ the insulinoma is underlined as the best technique for extirpation of this type of benign tumor, obtaining through it a high percentage of svmptomatic improvement.

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