Hypothalamic–pituitary–gonadal axis in which the hypothalamus and pituitary direct neuroendocrine function. The hypothalamus is located in the brain and secretes GnRH. GnRH travels down the anterior portion of the pituitary via the hypophyseal portal system and binds to receptors on the secretory cells of the adenohypophysis. In response to GnRH stimulation these cells produce LH and FSH, which travel into the blood stream. These two hormones play an important role in communicating to the gonads. In females FSH and LH act primarily to activate the ovaries to produce estrogen and inhibin and to regulate the menstrual cycle and ovarian cycle. Estrogen forms
Pituitary adenoma showed that acromegaly cases with "AIP" mutations occurred about 20 years before acromegaly cases without "AIP" mutations and these tumors are large and relatively treatment resistant. Due to their young age at onset, "AIP" mutations are the most frequent genetic cause of pituitary gigantism (29% of cases). X-LAG is a rare syndrome of very early childhood onset pituitary tumors/hyperplasia that leads to growth hormone excess and severe overgrowth and pituitary gigantism. Three FIPA families with X-LAG have been reported to date all of which had transmission of a chromosome Xq26.3 duplication from affected mother to affected son . The disease