Hyperpituitarism is a disease caused by pituitary gland hypersecretion, most commonly due to adenoma, leading to a series of abnormalities such as hyperthyroidism, hypothyroidism, hypogonadism, or hyperadrenalism.
Presentation
Hyperpituitarism causes two categories of symptoms: some caused by abnormal hormone secretion and others induced by the tumor itself. The latter consist of headaches and visual impairment, caused by optic nerve compression, or even hypopituitarism. If prolactin hypersecretion occurs in a pubertal girl, she will present with pubertal arrest. Galactorrhea may also be present in this case. Pubertal boys and prepubertal children complain about growth failure, visual impairment, and headaches, but the first category also experiences pubertal arrest [1]. Medical history and physical examination reveal menstrual abnormalities, acanthosis nigricans, and gynecomastia, in addition to other traits of the disease the patients notice.
Excess growth hormone causes acromegaly in adults and gigantism in children. Gigantism presents as accelerated complete growth in children, whereas acromegaly is characterized by the growth of the extremities (fingers and toes) and facial coarsening [2], as well as occlusion changes and apertognathia [3] [4]. Additional features include macrocephaly, prognathism, frontal bossing, irregular menses in women, and hyperhidrosis. The distance between teeth increases and the lips become thicker and darker [5]. In adult patients, cardiac hypertrophy sets in during the progression of the disease. Uterine, prostate, and colon benign tumors seem to have an increased incidence in these patients. The physician must also keep in mind the fact that these patients are prone to develop diabetes mellitus and peripheral neuropathies, as well as muscular and articular disorders, and conduct an active surveillance in this area [6] [7].
If the adrenocorticotropic hormone (ACTH) is involved, patient present with symptoms of excessive glucocorticoid secretion: hypertension, hirsutism, weight gain, acne, fatigability, and sleep apnea signs [8]. The clinician is often able to highlight the presence of the typical Cushing facies, as well as a dorsal cervical fat pad and bruising.
Workup
Hyperpituitarism workup should concentrate on detecting abnormal pituitary gland function based on whatever clinical findings the physician encounters. For instance, if hyperprolactinemia is suspected, serum prolactin should be measured several times if the obtained values are normal, but one determination is sufficient if a value above 200 ng/mL is found. A thyrotropin-releasing hormone (TRH) stimulation test can also be performed if a doubt exists, given that intravenous administration of TRH leads to an abrupt increase of prolactinemia 15 to 30 minutes later in normal individuals. Prolactinoma patients usually do not show this increase in prolactin serum levels.
A patient with an adrenocorticotropic hormone (ACTH) - releasing adenoma should undergo a urinary free cortisol excretion measurement, as well as a plasma cortisol determination. The first test accurately measures the cortisol secretion rate, while the second one needs to be interpreted based on the time the blood sample was drawn, keeping in mind that cortisol levels are normally highest from 6 to 8 AM and decline during the day. The lack of variation suggests Cushing disease. Additional investigations include the dexamethasone suppression testing and the corticotropin-releasing hormone (CRH) stimulation testing in uncertain cases.
When growth hormone oversecretion is suspected, the following tests should be ordered: serum insulin-like growth factor-I (IGF-I) measurement, serum insulin-like growth factor-binding protein-3 (IGFBP-3) dosage, and an oral glucose tolerance test (OGTT). After 1.75-g/kg oral glucose ingestion (maximum 75g), the serum growth hormone level should decrease to less than 5 ng/dL within 3 hours in healthy individuals.
The presence of the pituitary adenoma itself may be revealed by magnetic resonance imaging (MRI) with gadolinium administration, what is considered to be the gold standard [9] [10].
Treatment
Treatment for hyperpituitarism depends on the underlying cause and the specific hormones involved. Options may include medication to control hormone production, surgery to remove pituitary tumors, or radiation therapy. Medications such as dopamine agonists can be effective in reducing prolactin levels, while somatostatin analogs may be used to control growth hormone production. In some cases, hormone replacement therapy may be necessary if treatment affects normal hormone levels.
Prognosis
The prognosis for individuals with hyperpituitarism varies based on the cause and effectiveness of treatment. With appropriate management, many patients can lead normal, healthy lives. However, untreated hyperpituitarism can lead to serious complications, including cardiovascular disease, diabetes, and osteoporosis. Early diagnosis and treatment are crucial for improving outcomes and preventing long-term health issues.
Etiology
Hyperpituitarism is often caused by pituitary adenomas, which are benign tumors of the pituitary gland. These tumors can lead to the overproduction of hormones. Other causes may include genetic mutations, such as those seen in multiple endocrine neoplasia type 1 (MEN1), or conditions affecting the hypothalamus, which regulates pituitary function.
Epidemiology
Hyperpituitarism is relatively rare, with pituitary adenomas occurring in approximately 10-15% of the population. However, not all adenomas cause symptoms or require treatment. The condition can affect individuals of any age but is most commonly diagnosed in adults between the ages of 30 and 50. There is no significant gender predilection, although certain types of hormone overproduction may be more common in one gender.
Pathophysiology
The pathophysiology of hyperpituitarism involves the excessive secretion of one or more pituitary hormones. This overproduction can disrupt the normal balance of hormones in the body, leading to a range of symptoms and complications. For example, excess growth hormone can cause tissue overgrowth, while excess prolactin can interfere with reproductive functions. The underlying cause is often a pituitary adenoma, which can alter the normal regulatory mechanisms of hormone production.
Prevention
There are no specific measures to prevent hyperpituitarism, as the condition is often related to genetic factors or spontaneous tumor development. However, regular medical check-ups and monitoring of symptoms can aid in early detection and treatment. Maintaining a healthy lifestyle, including a balanced diet and regular exercise, may help reduce the risk of complications associated with the condition.
Summary
Hyperpituitarism is a condition characterized by the overproduction of hormones by the pituitary gland, often due to benign tumors. It can lead to a variety of symptoms and health issues, depending on the hormones involved. Diagnosis involves clinical evaluation, hormone testing, and imaging studies. Treatment options include medication, surgery, and radiation therapy. Early diagnosis and treatment are crucial for preventing complications and improving patient outcomes.
Patient Information
If you have been diagnosed with hyperpituitarism, it's important to work closely with your healthcare provider to manage the condition. Treatment plans are tailored to your specific needs and may involve medication, surgery, or other interventions. Regular follow-up appointments are essential to monitor your hormone levels and adjust treatment as necessary. Understanding your condition and staying informed about your treatment options can help you maintain a good quality of life.
References
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- Lima DL, Montenegro RM, Jr, Vieira AP, et al. Absence of periodontitis in acromegalic patients. Clin Oral Investig. 2009;13:165–169.
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- Chaudhary V, Bano S. Imaging of pediatric pituitary endocrinopathies. Indian J Endocrinol Metab. 2012;16(5):682-691.
- Lonser RR, Ksendzovsky A, Wind JJ, et al. Prospective evaluation of the characteristics and incidence of adenoma-associated dural invasion in Cushing disease. J Neurosurg. 2012;116(2):272-279.