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INTRODUCTION

The anterior pituitary is often referred to as the “master gland” because, together with the hypothalamus, it orchestrates the complex regulatory functions of multiple other glands (Fig. 168-1). The anterior pituitary produces six major hormones: (1) prolactin (PRL), (2) growth hormone (GH), (3) adrenocorticotropin hormone (ACTH), (4) luteinizing hormone (LH), (5) follicle-stimulating hormone (FSH), and (6) thyroid-stimulating hormone (TSH). Pituitary hormones are secreted in a pulsatile manner, reflecting intermittent stimulation by specific hypothalamic-releasing factors. Each of these pituitary hormones elicits specific responses in peripheral target glands. The hormonal products of these peripheral glands, in turn, exert feedback control at the level of the hypothalamus and pituitary to modulate pituitary function. Disorders of the pituitary include neoplasms or other lesions (granulomas, hemorrhage) that lead to mass effects and clinical syndromes due to excess or deficiency of one or more pituitary hormones.

FIGURE 168-1

Diagram of pituitary axes. Hypothalamic hormones regulate anterior pituitary tropic hormones that, in turn, determine target gland secretion. Peripheral hormones feedback to regulate hypothalamic and pituitary hormones. ACTH, adrenocorticotropin hormone; CRH, corticotropin-releasing hormone; FSH, follicle-stimulating hormone; GH, growth hormone; GHRH, growth hormone–releasing hormone; GnRH, gonadotropin-releasing hormone; IGF, insulin-like growth factor; LH, luteinizing hormone; PRL, prolactin; SRIF, somatostatin, somatotropin release–inhibiting factor; TRH, thyrotropin-releasing hormone; TSH, thyroid-stimulating hormone.

PITUITARY TUMORS

Pituitary adenomas are benign monoclonal tumors that arise from one of the five anterior pituitary cell types and may cause clinical effects from either overproduction of a pituitary hormone or compressive/destructive effects on surrounding structures, including the hypothalamus, pituitary, optic chiasm, and cavernous sinus. About one-third of all adenomas are clinically nonfunctioning and produce no distinct clinical hypersecretory syndrome. Among hormonally functioning neoplasms, tumors secreting PRL is the most common (~50%); they have a greater prevalence in women than in men. GH- and ACTH-secreting tumors each account for about 10–15% of functioning pituitary tumors. Adenomas are classified as microadenomas (<10 mm) or macroadenomas (≥10 mm). Pituitary adenomas (especially PRL- and GH-producing tumors) may be part of genetic familial syndromes such as MEN 1, Carney syndrome, or mutant aryl hydrocarbon receptor inhibitor protein (AIP) syndrome. Other entities that can present as a sellar mass include craniopharyngiomas, Rathke’s cleft cysts, sella chordomas, meningiomas, pituitary metastases, gliomas, and granulomatous disease (e.g., histiocytosis X, sarcoidosis).

Clinical Features

Symptoms from mass effects include headache; visual loss through compression of the optic chiasm superiorly (classically a bitemporal hemianopia); and diplopia, ptosis, ophthalmoplegia, and decreased facial sensation from cranial nerve compression laterally. Pituitary stalk compression from the tumor may also result in mild hyperprolactinemia. Symptoms of hypopituitarism or hormonal excess may be present as well (see below).

Pituitary apoplexy, typically resulting from hemorrhage into a preexisting adenoma or post-partum as Sheehan’s syndrome, is an endocrine emergency that typically presents with features that include severe ...

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