SDH-related pheochromocytoma and paraganglioma.
Best Pract Res Clin Endocrinol Metab
; 24(3): 415-24, 2010 Jun.
Article
en En
| MEDLINE
| ID: mdl-20833333
ABSTRACT
Pheochromocytoma and paraganglioma are rare tumors of adrenals as well as the sympathetic and parasympathetic paraganglia. Clinical presentation of these tumors depends on localization, secretory profile and malignant potential. Four distinct syndromes--PGL1-4--are related to mutations in the succinate dehydrogenase gene--mitochondrial complex involved in electron transfer and Krebs cycle. Here we describe etiology, genetics, as well as clinical aspects of SDH-related tumors. We also describe recent discoveries in HIF-related pathway of tumorigenesis and mutations in new SDH-related genes that have improved our understanding of this disease.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Paraganglioma
/
Feocromocitoma
/
Succinato Deshidrogenasa
/
Neoplasias de las Glándulas Suprarrenales
Límite:
Humans
Idioma:
En
Revista:
Best Pract Res Clin Endocrinol Metab
Asunto de la revista:
ENDOCRINOLOGIA
/
METABOLISMO
Año:
2010
Tipo del documento:
Article
País de afiliación:
Estados Unidos