Pituitary Tumour
Disease Details
Family Health Simplified
- Description
- A pituitary tumor is an abnormal growth in the pituitary gland, which can affect hormone levels and various bodily functions.
- Type
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Type: Pituitary tumors can be classified as benign adenomas or, less commonly, malignant carcinomas. They are also classified based on the hormones they secrete, such as prolactinomas, somatotroph adenomas, corticotroph adenomas, thyrotroph adenomas, and non-functioning adenomas.
Type of genetic transmission: Most pituitary tumors are sporadic and not inherited. However, a small percentage may occur as part of hereditary syndromes, such as Multiple Endocrine Neoplasia type 1 (MEN1), Carney complex, or familial isolated pituitary adenomas (FIPA). These hereditary conditions follow an autosomal dominant pattern of transmission. - Signs And Symptoms
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### Signs and Symptoms of Pituitary Tumors
1. **Hormonal Imbalances:**
- **Hyperprolactinemia:** Irregular menstrual cycles, galactorrhea (milk production), infertility in women, erectile dysfunction, and decreased libido in men.
- **Growth Hormone Excess:** Acromegaly (enlarged hands, feet, and facial features) or gigantism in children.
- **Cushing's Disease:** Central weight gain, purple stretch marks, high blood pressure, and high blood sugar.
- **Thyroid-Stimulating Hormone (TSH) Excess:** Hyperthyroidism symptoms like weight loss, rapid heartbeat, and nervousness.
2. **Mass Effects:**
- **Headaches:** Due to pressure on surrounding tissues.
- **Visual Disturbances:** Often bitemporal hemianopsia (loss of peripheral vision), due to compression of the optic chiasm.
- **Other Cranial Nerve Deficits:** Depending on tumor size and location.
3. **General Symptoms:**
- Fatigue
- Weakness
- Unexplained weight changes
- Nausea and vomiting
4. **Symptoms of Hypopituitarism:**
- Fatigue
- Weight gain or loss
- Sensitivity to cold
- Decreased appetite
- Low blood pressure
Prompt diagnosis and treatment are crucial to managing these symptoms effectively. - Prognosis
- Prognosis for pituitary tumors generally depends on several factors, including the type of tumor, size, whether it's benign or malignant, its location, and how much it has affected surrounding structures. Many pituitary tumors are benign and can be effectively treated or managed, leading to a good long-term outlook. However, some may cause significant hormonal imbalances or compress nearby optic nerves, impacting vision, which can complicate prognosis. In rare cases where the tumor is malignant, prognosis varies based on the extent of spread and response to treatment. Early detection and treatment usually result in better outcomes.
- Onset
- The onset of a pituitary tumor can vary greatly depending on the type, size, and growth rate of the tumor. Symptoms may develop slowly over time or more rapidly if the tumor grows quickly. Initial signs often stem from hormone imbalances caused by excess or insufficient hormone production. The onset usually includes symptoms such as headaches, vision problems, unexplained fatigue, and changes in menstrual cycles or sexual function.
- Prevalence
- The prevalence of pituitary tumors is estimated to be about 1 in 10,000 people. These tumors are relatively common, with studies suggesting that around 10-20% of the general population may have a pituitary tumor, although many are asymptomatic and go undiagnosed.
- Epidemiology
- Pituitary tumors are relatively common, accounting for about 10-15% of all intracranial tumors. These tumors can occur at any age but are most commonly diagnosed in adults, particularly between the ages of 30 and 60. They tend to be more frequently found in women than in men. Despite their prevalence, many pituitary tumors are asymptomatic and often go undiagnosed.
- Intractability
- Pituitary tumors are generally not considered intractable. Many can be successfully treated with surgery, medication, and/or radiation therapy, depending on their type, size, and location. However, some cases may be more challenging to treat due to the tumor's proximity to critical brain structures or its resistance to standard treatments. Regular monitoring and individualized treatment plans are essential for effective management.
- Disease Severity
- The severity of a pituitary tumor can vary widely depending on its size, type, and whether it is producing excess hormones. Some pituitary tumors are benign (non-cancerous) and may cause minimal or no symptoms, while others can lead to significant health issues, including hormonal imbalances, vision problems, and other neurological symptoms. The overall impact on health can range from mild to potentially life-threatening, especially if the tumor compresses vital brain structures or significantly disrupts hormone levels.
- Healthcare Professionals
- Disease Ontology ID - DOID:1785
- Pathophysiology
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Pathophysiology:
Pituitary tumors arise from the pituitary gland, a small organ located at the base of the brain that is responsible for hormone production and regulation. These tumors can be classified into functioning (hormone-producing) or non-functioning (non-hormone-producing) types. The pathophysiology involves abnormal growth of pituitary cells, which can result from genetic mutations, loss of tumor suppressor genes, or aberrant signaling pathways. Functioning pituitary tumors can cause conditions such as Cushing's disease, acromegaly, or prolactinoma, depending on the hormone that is excessively produced. Non-functioning tumors often grow large enough to cause symptoms by compressing nearby structures, leading to headaches, vision problems, or hypopituitarism due to impaired hormone production from the pressure exerted on the normal pituitary gland. - Carrier Status
- Pituitary tumors are typically not inherited, meaning there is no carrier status associated with them. They usually develop sporadically due to mutations that occur during a person's lifetime.
- Mechanism
- The pituitary gland or hypophysis is often referred to as the "master gland" of the human body. Part of the hypothalamic-pituitary axis, it controls most of the body's endocrine functions via the secretion of various hormones into the circulatory system. The pituitary gland is located below the brain in a depression (fossa) of the sphenoid bone known as the sella turcica. Although anatomically and functionally connected to the brain, the pituitary gland sits outside the blood–brain barrier. It is separated from the subarachnoid space by the diaphragma sella, therefore the arachnoid mater and thus cerebral spinal fluid cannot enter the sella turcica.The pituitary gland is divided into two lobes, the anterior lobe (which accounts for two thirds of the volume of the gland), and the posterior lobe (one third of the volume) separated by the pars intermedia.The posterior lobe (the neural lobe or neurohypophysis) of the pituitary gland is not, despite its name, a true gland. The posterior lobe contains axons of neurons that extend from the hypothalamus to which it is connected via the pituitary stalk. The hormones vasopressin and oxytocin, produced by the neurons of the supraoptic and paraventricular nuclei of the hypothalamus, are stored in the posterior lobe and released from axon endings (dendrites) within the lobe.The pituitary gland's anterior lobe (adenohypophysis) is a true gland which produces and secretes six different hormones: thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), follicle-stimulating hormone (FSH), luteinizing hormone (LH), growth hormone (GH), and prolactin (PRL).
- Treatment
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Treatment options depend on the type of tumor and on its size:
Prolactinomas (microadenomas and macroadenomas) are most often treated with cabergoline or bromocriptine (both dopamine agonists) as the first line of treatment. Medical treatment usually effectively decreases tumor size as well as alleviates symptoms. Pituitary adenomas are also followed by serial imaging (usually MRI) to detect any increase in size or mass effect on nearby structures. If medical therapy fails, the second option is usually transphenoidal pituitary surgery. A third line therapy is radiation therapy, proton therapy to shrink the tumor.
Thyrotropinomas are treated with transsphenoidal pituitary surgery as the first line treatment option. Medical therapy is a second line treatment, and thyrotropinomas respond to treatment with somatostatin receptor ligands such as octreotide or lanreotide. In people with thyrotropinomas, treatment with somatostatin receptor ligands normalized thyroid hormone levels in 80-90% of people, and 42% of people had a decreased tumor size.
Somatotrophic adenomas are primarily treated with transsphenoidal pituitary surgery, especially if symptoms of acromegaly are present. A second line treatment strategy (which can be used if there is a persistent or recurrent mass or symptoms after surgery) utilizes medical therapy, including octreotide or lanreotide, which are long-acting somatostatin analogs. These somatostatin receptor analogs inhibit secretion of growth hormone. They were found to be about 50–55% effective in reducing tumor mass and reducing growth hormone and insulin like growth factor 1 (IGF-1) levels in studies. The growth hormone receptor antagonist pegvisomant is also used in the treatment of somatotrophic adenomas. Pegvisomant blocks the action of growth hormone. It can either be used as monotherapy or combined with a somatostatin analog.
Corticotropinomas are primarily treated with transsphenoidal pituitary surgery, especially if signs and symptoms of Cushing syndrome are present. Medication therapy is second line, and includes steroidogenesis inhibitors (ketoconazole, metyrapone, osilodrostat, etomidate or mitotane) which decrease production of cortisol, pituitary blockers such as somatostatin receptor ligand pasireotide or the dopamine agonist cabergoline, or the glucocorticoid receptor antagonist mifepristone. These medications can be combined for a synergistic effect. Medication therapy is often used in conjunction with radiation therapy for corticotropinomas.
Surgery is a common treatment for pituitary tumors. The normal approach is trans-sphenoidal adenectomy, which usually can remove the tumor without affecting the brain or optic nerves.
Radiation is also used to treat pituitary adenomas. Examples include external beam or proton beam radiation therapy or stereotactic radiosurgery. External radiation of pituitary adenomas can arrest tumor growth for several years but pituitary failure develops within 10 years in most patients necessitating lifelong hormone replacement. Radiation therapy for pituitary adenomas is associated with a four-fold increase in mortality due to cerebrovascular disease. Lifelong monitoring of pituitary hormones is recommended after radiation therapy as hypopituitarism developed in 17% of those undergoing radiation therapy. - Compassionate Use Treatment
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For pituitary tumors, compassionate use treatment, as well as off-label or experimental treatments, may be considered under specific circumstances.
**Compassionate Use Treatment:**
Compassionate use, or expanded access, allows patients with serious or life-threatening conditions who lack other treatment options to access investigational drugs or therapies outside of clinical trials. For pituitary tumors, this might include access to novel targeted therapies, immunotherapies, or other drugs still under investigation that have shown promise in early clinical trials.
**Off-Label Treatments:**
Off-label use refers to the prescription of drugs for an indication that has not been approved by regulatory authorities. For pituitary tumors, off-label treatments might include:
- Use of certain chemotherapeutic agents or targeted therapies that are not specifically approved for pituitary adenomas but have shown efficacy in related cancers.
- Some hormone therapies or dopamine agonists (like cabergoline or bromocriptine), although these are often standard treatments, they might sometimes be used in a non-approved context for specific tumor types or treatment stages.
**Experimental Treatments:**
Experimental treatments for pituitary tumors may involve:
- Clinical trials investigating new drugs, biological agents, or combination therapies.
- Gene therapy targeting specific mutations associated with pituitary tumors.
- Novel radiotherapy techniques, such as proton beam therapy, being evaluated for their precision and reduced side effects.
Each case is unique, and the decision to pursue compassionate use, off-label, or experimental treatments should involve a thorough discussion between the patient and their healthcare provider, considering the potential benefits and risks. - Lifestyle Recommendations
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For individuals with a pituitary tumor, specific lifestyle recommendations may include:
1. **Regular Monitoring:** Regular follow-up appointments with healthcare providers to monitor hormone levels and tumor size.
2. **Balanced Diet:** Eating a nutritious and balanced diet to support overall health and well-being.
3. **Adequate Sleep:** Ensuring good quality and adequate sleep for physical and mental health.
4. **Stress Management:** Techniques such as yoga, meditation, or deep-breathing exercises to manage stress.
5. **Physical Activity:** Engaging in regular physical exercise, as tolerated and recommended by healthcare providers.
6. **Medication Compliance:** Adhering to prescribed medications and treatment plans to manage symptoms and hormone imbalances.
7. **Avoiding Alcohol and Tobacco:** Reducing or eliminating the consumption of alcohol and tobacco to improve overall health.
8. **Education:** Understanding the condition and being aware of the signs and symptoms of potential complications.
Consulting with healthcare providers for personalized advice is essential. - Medication
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For pituitary tumors, medication options depend on the type of tumor and the hormones it produces. Common medications include:
1. **Dopamine agonists** (e.g., cabergoline, bromocriptine) for prolactinomas to reduce prolactin levels and shrink the tumor.
2. **Somatostatin analogs** (e.g., octreotide, lanreotide) for growth hormone-secreting tumors to reduce hormone production.
3. **Pegvisomant** for acromegaly to block the effects of growth hormone.
4. **Ketoconazole, metyrapone, or mitotane** for Cushing's disease to inhibit cortisol production.
Treatment should be guided by an endocrinologist based on specific tumor characteristics. - Repurposable Drugs
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Research into the repurposing of existing drugs for the treatment of pituitary tumors is ongoing. Some drugs used for other conditions may show promise in this area. Examples include:
1. **Cabergoline and Bromocriptine:** Originally used for prolactinomas.
2. **Octreotide and Lanreotide:** Initially developed for acromegaly; may be useful in certain types of pituitary tumors.
3. **Temozolomide:** Originally for glioblastoma, showing effectiveness in aggressive pituitary tumors.
Consultation with a healthcare professional or oncologist is essential for appropriate diagnosis and treatment. - Metabolites
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For pituitary tumors, the study of metabolites can help in understanding the metabolic alterations associated with the tumor. Key metabolites often investigated include:
1. **Hormones**: Abnormal levels of pituitary hormones such as prolactin, ACTH (adrenocorticotropic hormone), growth hormone, and TSH (thyroid-stimulating hormone) are common indicators.
2. **Amino Acids**: Alterations in amino acid profiles, such as changes in levels of phenylalanine, tyrosine, and tryptophan, can be detected.
3. **Lactate**: Elevated lactate levels may be present due to altered glycolytic activity in the tumor.
4. **Lipids**: Changes in lipid metabolism might be evident, including variations in levels of cholesterol and fatty acids.
Metabolomics can provide a comprehensive profile of these metabolites, aiding in diagnosis, understanding the tumor microenvironment, and potentially guiding treatment strategies. - Nutraceuticals
- Currently, there isn't sufficient evidence to definitively state the efficacy of nutraceuticals in the treatment or management of pituitary tumors. Most treatment protocols for pituitary tumors focus on surgery, medication, and radiation therapy. Nutraceuticals might support general health but should not replace conventional treatments. Always consult a healthcare professional before starting any new supplement.
- Peptides
- Pituitary tumors, also known as pituitary adenomas, can be endocrine-active or inactive. Endocrine-active tumors secrete excess hormones, often peptides like prolactin, growth hormone, or adrenocorticotropic hormone (ACTH). These tumors can lead to various endocrine disorders depending on the type of hormone secreted. Peptides play a crucial role in the pathological manifestations of these tumors by disrupting normal hormonal balance. Nan refers to nanotechnology, which is being explored for its potential in diagnosis and treatment, such as targeted drug delivery or imaging to improve outcomes for patients with pituitary tumors.