Hyperpituitarism Clinical Presentation
- Author: Alicia Diaz-Thomas, MD, MPH; Chief Editor: Stephen Kemp, MD, PhD more...
The clinical presentation of a pituitary adenoma primarily results from the oversecreted hormone. The tumor mass itself may cause headaches, visual changes due to optic nerve compression, or hypopituitarism.
The presentation of prolactinomas may vary, depending on the age and sex of the child.
Prepubertal children typically present with a combination of headache, visual disturbance, and growth failure.
Pubertal females frequently present with symptoms of pubertal arrest or hypogonadism (with or without galactorrhea) due to suppression of gonadotropin secretion or local compression of the pituitary.
Pubertal males may present with headaches, visual impairment, and pubertal arrest or growth failure.
Excess adrenocorticotropic hormone
The most sensitive indicator of excess glucocorticoid secretion in children is weight gain with concurrent growth failure, which generally precedes other manifestations.
Patients commonly present with weight gain that tends to be generalized rather than centripetal.
Hirsutism and premature adrenarche may occur in prepubertal children.
Hypertension may be present.
Pubertal arrest, acne, fatigue, and depression are also common.
Snoring, poor sleep quality, deteriorating academic performance (compared with prior school terms), or other signs of obstructive sleep apnea (OSA) should prompt a formal sleep study and consultation with a pulmonologist.
Excess growth hormone
The presentation of gigantism in a child is usually dramatic, unlike the insidious onset of acromegaly in adults.
The cardinal clinical feature of gigantism is longitudinal growth acceleration secondary to GH excess.
Presentation depends on whether the epiphyseal growth plate is open. Before epiphyseal fusion, accelerated growth velocity is prominent. As epiphyseal fusion approaches, the spectrum of symptoms resembles the presentation in adults (eg, coarsening of facial features, change in ring and shoe size).
Prolactinoma may include the following:
Hypogonadism, leading to pubertal arrest, pubertal failure, or pubertal delay
Menstrual abnormalities, including primary or secondary amenorrhea
Cushing disease may include the following (see images below):
See the list below:
Cushingoid appearance includes a dorsal cervical fat pad, moon facies, bruising, and striae. These features are only observed in patients with advanced long-standing disease.
Growth failure and short stature may be observed.
Weight gain and obesity in children with Cushing disease tends to be generalized rather than centripetal.
Pubertal arrest, failure, or delay may occur.
Amenorrhea may be noted.
Hypertension may be present.
In patients with gigantism, all growth parameters are affected, although not necessarily symmetrically. GH excess over time is characterized by progressive cosmetic disfigurement and systemic organ manifestations. The following may be noted:
Mild-to-moderate obesity (common)
Macrocephaly, which may precede linear growth
Exaggerated growth of the hands and feet with thick fingers and toes
Coarse facial features, including frontal bossing and prognathism
Peripheral neuropathies (eg, carpal tunnel syndrome)
Cardiovascular disease: Prolonged GH excess can result in cardiac hypertrophy, hypertension, and left ventricular hypertrophy.
Tumors: Although benign tumors, including uterine myomas, prostatic hypertrophy, colon polyps, and skin tags, may be frequently encountered in acromegaly, the documentation of the overall prevalence of malignancies in patients with acromegaly remains controversial.
Endocrinopathies: Frequently associated endocrinopathies include hypogonadism, diabetes, decreased glucose tolerance, and hyperprolactinemia. OSA has been reported in up to half of patients with acromegaly, particularly those who are obese or older than 50 years.
Hypothalamic dysfunction can promote tumor growth, but overwhelming evidence points to intrinsic pituicyte genetic disruption as the main underlying cause of pituitary tumorigenesis. The monoclonal nature of most pituitary adenomas, confirmed with X-inactivation studies, implies their origin from a clonal event in a single cell. Most pituitary adenomas are functional, and clinical presentation typically depends on the particular pituitary hormone that is hypersecreted. Nonfunctioning pituitary adenomas are rare in children, accounting for only 3-6% of all adenomas in 2 large series; they comprise 30% of adenomas in adults.
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