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Prolactinoma

Disease Details

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Description: Prolactinoma is a noncancerous tumor of the pituitary gland that overproduces the hormone prolactin, leading to various symptoms including reproductive and hormonal imbalances.
Type: Prolactinoma is a benign tumor of the pituitary gland. Most prolactinomas occur sporadically and are not inherited. However, in rare cases, they can be associated with a genetic condition called Multiple Endocrine Neoplasia type 1 (MEN1), which follows an autosomal dominant pattern of inheritance.
Signs And Symptoms: The most common symptoms at the time of diagnosis often differ between males and females. Women tend to experience more symptoms related directly to prolactin levels, such as amenorrhea and galactorrhea. By contrast, men frequently exhibit fertility issues and hormonal disruptions, such as a loss of libido, but are also more likely to show signs of the tumor compressing their brain tissue, such as headaches, vision changes, and other symptoms outlined below.The symptoms due to a prolactinoma are broadly divided into those that are caused by increased prolactin levels or mass effect. Those that are caused by increased prolactin levels are:
Amenorrhea (disappearance of ovulation periods)
Galactorrhea (Milk production; infrequent in men)
Loss of axillary and pubic hair
Hypogonadism (Reduced function of the gonads.)
Gynecomastia (an increase in male breast size)
Erectile dysfunction (in males)Those that are caused by mass effect are:
Headaches
Vision Changes-visual field deficits, blurred vision, decreased visual acuity
Cranial nerve palsies-especially with invasive tumors or with pituitary apoplexy
Seizures, Hydrocephalus, Unilateral exophthalmos are rare presentations
Hypopituitarism
Pituitary apoplexy is a medical emergency because of spontaneous hemorrhage into the pituitary tumor and presents with severe headaches, vision changes, and acute panhypopituitarism.
Prognosis: People with microprolactinoma generally have an excellent prognosis. In 95% of cases, the tumor will not show any signs of growth after a 4 to 6-year period.Macroprolactinomas often require more aggressive treatment otherwise they may continue to grow. There is no way to reliably predict the rate of growth, as it is different for every individual. Regular monitoring by a specialist to detect any major changes in the tumor is recommended.
Onset: Prolactinoma onset can vary; it may develop gradually over time, often during reproductive years. Symptoms can include headaches, vision problems, and hormonal imbalances, which can lead to irregular menstrual cycles in women and reduced libido in men.
Prevalence: Prolactinoma is a relatively common type of pituitary tumor. It is the most frequently occurring type of hormone-producing pituitary tumor. The prevalence of prolactinoma is estimated to be around 50 to 100 cases per 100,000 people, with higher rates observed in women, especially those of childbearing age.
Epidemiology: Autopsy studies indicate that 6-25% of the U. S. population have small pituitary tumors. Forty percent of these pituitary tumors produce prolactin, but most are not considered clinically significant. Clinically significant pituitary tumors affect the health of approximately 14 out of 100,000 people. In non-selective surgical series, this tumor accounts for approximately 25-30% of all pituitary adenomas. Some growth hormone (GH)–producing tumors also co-secrete prolactin. More than 90% of prolactinoma is microprolactinomas, which are much more common than macroprolactinomas.
Intractability: Prolactinoma is not typically considered intractable. It is a type of benign pituitary tumor that produces an excess amount of prolactin. Treatment options, such as medications (e.g., dopamine agonists like cabergoline and bromocriptine), surgery, and, in some cases, radiation therapy, are often effective in managing the condition. Most patients respond well to medical treatment, which can shrink the tumor and reduce prolactin levels.
Disease Severity: Prolactinoma is a benign (non-cancerous) tumor of the pituitary gland that produces an excess amount of prolactin. The severity of symptoms can vary widely:

1. **Mild**: Small tumors (microadenomas, less than 10 mm) may cause mild symptoms or none at all.
2. **Moderate**: Larger tumors (macroadenomas, more than 10 mm) can cause more significant hormonal imbalances and symptoms such as headaches, vision problems, and reproductive issues.
3. **Severe**: If left untreated, larger tumors can potentially cause severe complications, including persistent vision loss, significant hormone dysregulation, and even hypopituitarism (reduced hormone production from the pituitary gland).

Nano description not applicable to the answer context.
Healthcare Professionals: Disease Ontology ID - DOID:5394
Pathophysiology: Prolactinoma is a benign pituitary adenoma that overproduces prolactin. The pathophysiology involves the excessive secretion of prolactin, which can suppress gonadotropin-releasing hormone (GnRH) from the hypothalamus. This suppression leads to decreased secretion of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) from the anterior pituitary, resulting in hypogonadism. In premenopausal women, this can cause amenorrhea and galactorrhea, while in men and postmenopausal women, it can lead to symptoms like decreased libido and osteoporosis. The excess prolactin also affects the brain and can cause headaches and visual disturbances due to the tumor's mass effect.
Carrier Status: Prolactinoma is a benign tumor of the pituitary gland that produces an excess amount of prolactin. It is not typically associated with a carrier status or inherited in a traditional genetic manner. The condition often occurs sporadically, though in rare cases, it can be part of multiple endocrine neoplasia type 1 (MEN1), an inherited disorder. If prolactinoma is part of MEN1, genetic testing for MEN1 mutations may be considered. However, prolactinomas themselves do not generally have a "carrier status."
Mechanism: A prolactinoma is a benign pituitary tumor that produces an excessive amount of prolactin. This overproduction of prolactin can lead to various clinical symptoms, primarily related to reproductive and endocrine functions.

**Mechanism:**
1. **Hormonal Regulation:** Prolactin production is primarily inhibited by dopamine, which is released from the hypothalamus. The dopamine binds to D2 receptors on lactotroph cells in the anterior pituitary gland, suppressing prolactin secretion.
2. **Prolactinoma Development:** In prolactinomas, the normal regulatory mechanisms are disrupted, leading to hypersecretion of prolactin. The exact cause of these tumors is not well understood, but they result in increased levels of circulating prolactin.

**Molecular Mechanisms:**
1. **Genetic Mutations:** Some prolactinomas are associated with genetic mutations, notably in the MEN1 gene, which is responsible for multiple endocrine neoplasia type 1. Mutations in this gene can lead to tumor development in endocrine tissues, including the pituitary.
2. **Signaling Pathways:** Dysregulation in signaling pathways involving dopamine receptors (especially D2 receptors) can contribute to uncontrolled prolactin secretion. Alterations in intracellular signaling pathways, such as the cyclic AMP (cAMP) pathway and its downstream effectors, are also implicated.
3. **Cell Cycle Regulators:** Abnormalities in cell cycle regulation, involving cyclins and cyclin-dependent kinases (CDKs), can play a role in the pathogenesis of prolactinomas by promoting unchecked cell proliferation of lactotrophs.

The understanding of prolactinomas at the molecular level is still evolving, but these mechanisms provide insight into the development and potential treatment strategies for this condition.
Treatment: Prolactinoma, a benign tumor of the pituitary gland that overproduces prolactin, is typically managed through several treatment approaches:

1. **Medication**: Dopamine agonists such as cabergoline and bromocriptine are commonly prescribed, as they reduce prolactin levels and often shrink the tumor.

2. **Surgery**: If drug therapy is ineffective or not tolerated, transsphenoidal surgery may be performed to remove the tumor.

3. **Radiation Therapy**: This is considered when medication and surgery fail to control the tumor or prolactin levels.

Patient response to treatment is regularly monitored through blood tests for prolactin levels and imaging studies like MRI to assess tumor size.
Compassionate Use Treatment: For prolactinoma, compassionate use treatment may include the experimental use of therapies not yet fully approved by regulatory bodies. Off-label or experimental treatments might involve medications primarily approved for other conditions but shown to be effective in some cases of prolactinoma.

1. **Temozolomide**: This is an oral chemotherapy drug approved for certain types of brain tumors, which has been used off-label for aggressive or resistant prolactinomas.

2. **Pasireotide**: This is a somatostatin analog initially developed for Cushing's disease and acromegaly, used experimentally in prolactinoma cases resistant to standard therapy.

3. **Everolimus**: This is an mTOR inhibitor approved for certain cancers and used off-label for refractory pituitary tumors, including prolactinomas.

Patients seeking these treatments typically must do so under clinical trial settings or special compassionate use programs, regulated by respective health authorities.
Lifestyle Recommendations: For prolactinoma, consider the following lifestyle recommendations:

1. Regular Monitoring: Ensure routine follow-ups with your healthcare provider to monitor the tumor's size and hormone levels.
2. Diet: Maintain a balanced diet to support overall health; no specific diet is known to affect prolactinoma directly.
3. Exercise: Engage in regular physical activity to improve overall well-being and reduce stress.
4. Stress Management: Practice stress-reducing techniques such as yoga, meditation, or mindfulness to help manage symptoms.
5. Medication Adherence: Take prescribed medications as directed to control prolactin levels and manage symptoms.
6. Awareness: Know and report any changes in symptoms such as headaches, vision changes, or menstrual irregularities to your healthcare provider promptly.
7. Bone Health: Discuss calcium and vitamin D supplementation with your doctor, as prolactinoma can affect bone density.
Medication: Dopamine is the chemical that normally inhibits prolactin secretion, so clinicians may treat prolactinoma with drugs that act like dopamine such as bromocriptine and cabergoline. This type of drug is called a dopamine agonist. These drugs shrink the tumor and return prolactin levels to normal in approximately 80% of patients. Both bromocriptine and cabergoline have been approved by the Food and Drug Administration for the treatment of hyperprolactinemia. Bromocriptine is associated with side-effects such as nausea and dizziness and hypotension in patients with already low blood pressure readings. To avoid these side-effects, it is important for bromocriptine treatment to start slowly.Bromocriptine treatment should not be interrupted without consulting a qualified endocrinologist. Prolactin levels often rise again in most people when the drug is discontinued. In some, however, prolactin levels remain normal, so the doctor may suggest reducing or discontinuing treatment every two years on a trial basis. Recent studies have shown increased success in remission of prolactin levels after discontinuation, in patients having been treated for at least 2 years prior to cessation of bromocriptine treatment.Cabergoline is also associated with side effects such as nausea and dizziness, but these may be less common and less severe than with bromocriptine. However, people with low blood pressure should use caution when starting cabergoline treatment, as the long half-life of the drug (4–7 days) may inadvertently affect their ability to keep their blood pressure within normal limits, creating intense discomfort, dizziness, and even fainting upon standing and walking until the single first dose clears from their system. As with bromocriptine therapy, side effects may be avoided or minimized if treatment is started slowly. If a patient's prolactin level remains normal for 6 months, a doctor may consider stopping treatment. Cabergoline should not be interrupted without consulting a qualified endocrinologist.Other dopamine agonists that have been used less commonly to suppress prolactin include dihydroergocryptine, ergoloid, lisuride, metergoline, pergolide, quinagolide, and terguride.
Repurposable Drugs: Prolactinoma is a type of pituitary adenoma that produces an excess amount of prolactin. Repurposable drugs primarily target the dopamine D2 receptors to inhibit prolactin secretion. Some of the drugs that are commonly used and have been repurposed for treating prolactinomas include:

1. **Cabergoline** - A dopamine agonist with a long half-life that is effective in reducing prolactin levels and shrinking tumors.
2. **Bromocriptine** - Another dopamine agonist that is often used, though it may have more side effects compared to cabergoline.

These medications can be crucial in managing prolactin levels and reducing tumor size, often serving as first-line treatments for prolactinoma.
Metabolites: Prolactinoma is a benign tumor of the pituitary gland that produces an excessive amount of prolactin. Elevated prolactin levels can lead to various metabolic disturbances. Key metabolic effects include:

1. **Hyperprolactinemia**: Increased levels of prolactin in the blood.
2. **Hypogonadism**: Reduced levels of sex hormones (estrogen in women, testosterone in men) due to inhibition of gonadotropin-releasing hormone (GnRH).
3. **Bone Metabolism**: Prolonged hyperprolactinemia can lead to decreased bone mineral density and higher risk of osteoporosis due to reduced levels of sex hormones.
4. **Lipid Metabolism**: Increased prolactin levels may be associated with alterations in lipid metabolism, potentially leading to changes in cholesterol levels.

These metabolic effects can have significant clinical implications, necessitating appropriate management and treatment.
Nutraceuticals: There is limited evidence specifically supporting the use of nutraceuticals for the treatment of prolactinoma, a type of benign pituitary tumor that produces excessive amounts of prolactin. Conventional treatments typically include medications like dopamine agonists (e.g., bromocriptine or cabergoline), surgery, and radiation therapy. However, certain nutraceuticals may support overall health and hormonal balance:

1. **Vitamin D**: Adequate levels may support pituitary function and immune health.
2. **Omega-3 fatty acids**: Anti-inflammatory properties can support overall health.
3. **Zinc**: Essential for immune function and may have a role in hormonal regulation.

Always consult a healthcare provider before starting any new supplement regimen.
Peptides: For prolactinoma, peptides such as cabergoline and bromocriptine are commonly used as treatments. These dopamine agonists work by reducing prolactin secretion and, in most cases, help to shrink the tumor.

PC-1 (pituitary adenylate cyclase-activating polypeptide) is another peptide under research for its potential role in regulating pituitary function, including prolactin secretion, though it is not yet a standard treatment. Nan (not applicable) does not apply here since specific peptides are relevant to the condition.