Pituitary Hyperfunction – Top USMLE Diseases

Pituitary adenomas are tumors that develop within the anterior lobe of the pituitary gland. They are classified by size (either micro- or macroadenomas) and by their ability to secrete hormones. Non-functioning or non-secretory adenomas do not secrete hormones but can compress surrounding pituitary tissue, leading to hypopituitarism. Secretory adenomas secrete various hormones depending on the cell type from which they evolved, leading to hyperpituitarism.

Acromegaly and gigantism are caused by an excessive production of growth hormone (GH) by the pituitary gland. Tall stature is a clinical sign observed in some patients with gigantism. Increased GH and insulin-like growth factor-1 (IGF-1) are responsible for inducing hypersomatotropism. Acromegaly is usually produced by pituitary tumors secreting GH or, less commonly, by extrapituitary disorders. Gigantism typically results in children from excess GH before growth-plate closure; acromegaly typically results from excess GH after growth-plate closure. Diagnosis involves neuroimaging of the pituitary and laboratory tests to evaluate the hypothalamic-pituitary axis. Treatment depends on the operative status of the tumor (if present), or a nonoperative treatment strategy may be utilized.

Hormones are messenger molecules that are synthesized in one part of the body and move through the bloodstream to exert specific regulatory effects on another part of the body. Hormones play critical roles in coordinating cellular activities throughout the body in response to the constant changes in both the internal and external environments. The work of hormones allows the body to maintain homeostasis and regulate growth and development. Hormones are typically either made from amino acids or derived from cholesterol (the latter group being known as steroid hormones). Hormones exert their effects by binding to receptors either on the cell surface (most amino acid–based hormones) or within the cytosol (steroid hormones). Ultimately, binding to receptors triggers changes in gene expression or enzymatic activity within the cell.

The pituitary gland, also known as the hypophysis, is considered the “master endocrine gland” because it releases hormones that regulate the activity of multiple major endocrine organs in the body. The gland sits on the sella turcica, just below the hypothalamus, which is the primary regulator of the pituitary gland. The pituitary gland has 2 lobes: the anterior lobe (the adenohypophysis) and the posterior lobe (the neurohypophysis). Each lobe has its own regulation, set of secretory products, and feedback loops. Abnormalities in the pituitary gland can lead to a wide range of clinical conditions, some of which include hyperprolactinemia, acromegaly, hyperthyroidism or hypothyroidism, and central diabetes insipidus.

Hypopituitarism is a condition characterized by pituitary hormone deficiency. This condition primarily results from a disease of the pituitary gland, but it may arise from hypothalamic dysfunction. Pituitary tumors are one of the most common causes. The majority of cases affect the anterior pituitary lobe (adenohypophysis), which accounts for 80% of the gland. The hormones produced from this lobe are growth hormone, follicle-stimulating hormone, luteinizing hormone, thyroid-stimulating hormone (TSH), adrenocorticotropic hormone, and prolactin. When the posterior lobe (neurohypophysis) is also damaged, loss of antidiuretic hormone and oxytocin occurs. All of these hormones regulate the activities of different organs, and thus the effects of pituitary hypofunction are multisystemic. The diagnosis is made through a combination of clinical findings, hormone levels, provocation tests, and brain imaging. Treatment is hormone replacement and addressing the etiology.

Hyperprolactinemia is defined as a condition of elevated levels of prolactin (PRL) hormone in the blood. The PRL hormone is secreted by the anterior pituitary gland and is responsible for breast development and lactation. Many factors contribute to the development of hyperprolactinemia. The most common cause is PRL-secreting pituitary adenomas (prolactinomas). Diagnosis is achieved through hormonal testing to rule out other endocrine conditions and confirmatory imaging tests. Dopamine agonists are the 1st-line drugs for treatment. Refractory cases require surgery and possibly radiation therapy.