Neoplasia endocrina múltiple tipo 1 / Multiple endocrine neoplasia type 1
Rev. chil. endocrinol. diabetes
;
2(2): 108-114, abr. 2009. tab
Artigo
em Espanhol
| LILACS
| ID: lil-612494
ABSTRACT
Multiple endocrine neoplasia type 1 (NEM1) is an uncommon autosomal dominant disease caused by an alteration of menin, a tumor suppression protein and is characterized by the presence of primary tumors in at least two different endocrine tissues. It is described as the three P diseasesince it involves mainly the pituitary, parathyroid and pancreas. However more than 20 different tumor locations have been described. Most tumors are benign and primary hyperparathyroidism is the first manifestation of the disease in 90 percent of cases enteropancreatic tumors appear in approximately 60 percent of patients and pituitary adenomas, usually prolactinomas, in 30 percent. Skin lesions, non functional adrenal adenomas and neuroendocrine tumors such as carcinoid are also part of the disease. We describe the pathogenesis, clinical presentation, diagnosis and treatment of NEM1.
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Índice:
LILACS (Américas)
Assunto principal:
Neoplasia Endócrina Múltipla Tipo 1
Tipo de estudo:
Estudo diagnóstico
/
Estudo prognóstico
/
Estudo de rastreamento
Limite:
Humanos
Idioma:
Espanhol
Revista:
Rev. chil. endocrinol. diabetes
Assunto da revista:
Endocrinologia
Ano de publicação:
2009
Tipo de documento:
Artigo
País de afiliação:
Chile
Instituição/País de afiliação:
Pontificia Universidad Católica de Chile/CL
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