Article information
Abstract
Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary syndrome known to predispose subjects to endocrine neoplasms in a variety of tissues such as the parathyroid glands, the pituitary gland, and the gastrointestinal tract. We report the case of a male patient who, in addition to the traditionally described conditions (hyperparathyroidism and gastrinoma), was found to have other tumor lesions arising from both endocrine cells (insulinoma, gastric carcinoid, adrenal adenoma, and non-functional pancreatic neuroendocrine tumors), and non-endocrine cells (lipoma and collagenoma). The frequent recurrence of lesions in not completely resected susceptible tissues (as occurs in hyperparathyroidism and duodenal gastrinoma) as well as doubts concerning their clinical significance in MEN1 has raised some questions regarding the therapeutic management of such lesions, and this controversy is briefly reviewed.
Keywords:
Insulinoma
Gastrinoma
Hyperparathyroidism
Neuroendocrine tumors
Multiple endocrine neoplasia
Resumen
La neoplasia endocrina múltiple de tipo 1 (MEN1) es un síndrome hereditario raro conocido por la predisposición a la aparición de neoplasias endocrinas en distintos tejidos como paratiroides, hipófisis y tracto gastrointestinal. Se presenta el caso de un varón en el que además de manifestaciones tradicionalmente descritas (hipeparatiroidismo y gastrinoma) se objetivan otras lesiones tumorales procedentes de células de estirpe endocrinológica (insulinoma, carcinoide gástrico, adenoma suprarrenal, tumores neuroendocrino no funcionantes del páncreas) y no endocrinológica (lipoma y colagenoma). La frecuente recurrencia de las lesiones sobre los tejidos susceptibles no resecados en su totalidad (como en el caso del hiperparatiroidismo y del gastrinoma duodenal) y las dudas sobre su significado clínico en el MEN1 suscitan cierta controversia en la actualidad sobre las recomendaciones en el manejo terapéutico de dichas lesiones que se revisa brevemente.
Palabras clave:
Insulinoma
Gastrinoma
Hiperparatiroidismo
Tumores neuroendocrinos
Neoplasia endocrina múltiple
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