Genetic disorders and insulinoma/glucagonoma.
Endocr Relat Cancer
; 31(5)2024 May 01.
Article
en En
| MEDLINE
| ID: mdl-38552306
ABSTRACT
Insulinoma and glucagonoma are two rare functioning neoplasms of the neuroendocrine cells of the pancreas, respectively, characterized by an uncontrolled over-secretion of insulin or glucagon, responsible for the development of the hypoglycemic syndrome and the glucagonoma syndrome. They prevalently arise as sporadic tumors; only about 10% of cases develop in the context of rare inherited tumor syndromes, such as multiple endocrine neoplasia type 1 (MEN1), neurofibromatosis type 1 (NF1), and tuberous sclerosis complex (TSC), being the result of an autosomal-dominant germline heterozygous loss-of-function mutation in a tumor-suppressor gene. Here, we reviewed the main epidemiological and clinical aspects of insulinoma and glucagonoma in the context of genetic syndromes.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Pancreáticas
/
Neoplasia Endocrina Múltiple Tipo 1
/
Glucagonoma
/
Insulinoma
Límite:
Humans
Idioma:
En
Revista:
Endocr Relat Cancer
/
Endocr. relat. cancer
/
Endocrine-related cancer
Asunto de la revista:
ENDOCRINOLOGIA
/
NEOPLASIAS
Año:
2024
Tipo del documento:
Article
País de afiliación:
Italia
Pais de publicación:
Reino Unido