60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly

Abstract

Acromegaly (ACM) is a chronic, progressive disorder caused by the persistent hypersecretion of GH, in the vast majority of cases secreted by a pituitary adenoma. The consequent increase in IGF1 (a GH-induced liver protein) is responsible for most clinical features and for the systemic complications associated with increased mortality. The clinical diagnosis, based on symptoms related to GH excess or the presence of a pituitary mass, is often delayed many years because of the slow progression of the disease. Initial testing relies on measuring the serum IGF1 concentration. The oral glucose tolerance test with concomitant GH measurement is the gold-standard diagnostic test. The therapeutic options for ACM are surgery, medical treatment, and radiotherapy (RT). The outcome of surgery is very good for microadenomas (80-90% cure rate), but at least half of the macroadenomas (most frequently encountered in ACM patients) are not cured surgically. Somatostatin analogs are mainly indicated after surgical failure. Currently their routine use as primary therapy is not recommended. Dopamine agonists are useful in a minority of cases. Pegvisomant is indicated for patients refractory to surgery and other medical treatments. RT is employed sparingly, in cases of persistent disease activity despite other treatments, due to its long-term side effects. With complex, combined treatment, at least three-quarters of the cases are controlled according to current criteria. With proper control of the disease, the specific complications are partially improved and the mortality rate is close to that of the background population.

MANAGEMENT OF ENDOCRINE DISEASE: Acromegaly and cancer: an old debate revisited

“…Familial syndromes associated with acromegaly include multiple endocrine neoplasia type 1 (MEN 1), MEN 4, McCune-Albright, and Carney complex. Moreover, germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene and X-chromosomal microduplication have been linked to pituitary adenomas (1). There are also reports on acromegaly patients harboring several different tumors where a genetic defect was suspected but not identified (63).…”

“…It is a rare disease, typically caused by a GH-secreting pituitary adenoma, whose natural history has been modified in the past two decades by a combined therapeutic approach involving surgery, radiotherapy and pharmacological treatment (1).…”

Prescription patterns of long-acting somatostatin analogues

“…1–3 As a result, patients suffer body deformations and even cardiovascular and neuroendocrine complications that can alter their quality of life and life expectancy in addition to the increased costs from medical and surgical therapy. 4,5 …”

Recidiva de macroadenoma hipofisario posterior a tratamiento estándar

“…De igual forma Daly F. y colaboradores han estudiado la relación entre el síndrome de adenomas hipofisarios aislados, con una mutación en el gen de la proteína intercambiadora de hidrocarburos aromáticos 15 , por tanto existe una importante carga genética en el desarrollo de la acromegalia. Es importante resaltar que si la elevación en los niveles de GH se presenta antes del cierre metafisario de los huesos largos, se desarrollara gigantismo hipofisario 16 .…”