Update on the Genetics of Pituitary Tumors

• Autores: Sayka Barry, Márta Korbonits
• Localización: Endocrinology and metabolism clinics of North America, ISSN 0889-8529, Vol. 49, Nº. 3, 2020 (Ejemplar dedicado a: Pituitary Disorders), págs. 433-452
• Idioma: inglés
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• Resumen

  • The prevalence of symptomatic pituitary neuroendocrine tumors (PitNETs) is approximately 1 in 1000 in the general population. Most of these tumors occur sporadically and are not part of syndromic disorders. An estimated 5% of them are considered to have familial origin. There are a few well-defined genetic syndromes known to be associated with pituitary adenomas, including multiple endocrine neoplasia (MEN) type 1 and Carney complex (CNC), and nonsyndromic familial isolated pituitary adenomas (FIPA). PitNETs are slow-growing, usually benign tumors generally thought to be of monoclonal origin. However, they can cause considerable morbidity because of mass effects on surrounding tissues or altered hormonal secretion. Furthermore, tumors can invade into the surrounding structures leading to difficulties in complete surgical resection. Medical treatment is available with somatostatin analogues or dopamine agonist for some of the tumors, whereas for others surgery and radiotherapy are the only therapeutic options