Genetic disorders and insulinoma/glucagonoma.
Endocr Relat Cancer
; 31(5)2024 May 01.
Article
em 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.
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Base de dados:
MEDLINE
Assunto principal:
Neoplasias Pancreáticas
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Neoplasia Endócrina Múltipla Tipo 1
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Glucagonoma
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Insulinoma
Idioma:
En
Ano de publicação:
2024
Tipo de documento:
Article