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Multiple Endocrine Neoplasia - Types, Causes, Symptoms, Diagnosis, and Treatment

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Multiple Endocrine Neoplasia - Types, Causes, Symptoms, Diagnosis, and Treatment

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Multiple endocrine neoplasia is a condition that causes tumors in hormone-producing parts of the body. Read below to know more about the condition.

Medically reviewed by

Dr. Thiyagarajan. T

Published At July 18, 2022
Reviewed AtOctober 5, 2023

Introduction:

Multiple endocrine neoplasia (MEN) is a condition that causes the growth of tumors in the endocrine system, which comprises different glands that produce, store, and secrete hormones into the bloodstream. Hormones are chemical messengers released by the endocrine glands to carry out specific functions in different parts of the body. Growth of tumors occurs in different endocrine glands in MEN, leading to an excess hormone production from the affected glands, causing symptoms. The tumors may be benign (harmless) or malignant (cancerous). The condition affects about one in every 30,000 to 35,000 people.

What Are the Types of Multiple Endocrine Neoplasia (MEN)?

There are many types of MEN, type 1 and type 2 being the most common.

  • Multiple Endocrine Neoplasia Type 1:

MEN type 1 involves tumors occurring in the pancreas, parathyroid glands, and the pituitary glands. Commonly, the parathyroid glands are involved first, causing symptoms of overactive parathyroid glands that cause excess calcium to be released into the bloodstream. In persons with hyperparathyroidism, symptoms may not occur for many years. However, they may present with symptoms like thinning of bones, making them more prone to fractures, kidney stones, nausea and vomiting, high blood pressure, and weakness. People with a genetic trait for MEN type 1 tend to develop hyperparathyroidism by the age of 50.

  • Multiple Endocrine Neoplasia Type 2:

MEN type 2 is further classified into three subtypes, namely:

  1. Type 2A.
  2. Type 2B.
  3. Familial medullary thyroid carcinoma (FMTC).

About 90 percent of all the cases are of type 2A. People affected with MEN type 2 tend to develop familial medullary thyroid carcinoma (FTMC) irrespective of the subtype. People with type 2A can develop hyperparathyroidism, which causes an increase in blood calcium levels. People with type 2B can develop tumors in the nerve cells of the GI (gastrointestinal) tract, lips, eyelids, and tongue, which usually occur before ten years of age.

What Are the Causes of Multiple Endocrine Neoplasia (MEN)?

Both MEN type 1 and type 2 are inherited, which means that a defective gene passed down in families is the cause. MEN type 1 occurs due to an abnormality in the MEN1 (menin 1) gene, whereas MEN type 2 occurs due to an abnormality in RET (ret proto-oncogene) gene. Sometimes, a patient without a family history can develop MEN due to a new genetic change that develops due to unknown reasons.

Who Are at an Increased Risk of Developing MEN?

MEN is an inherited condition that is passed down in families. Either of the parents with a genetic mutation (changes in genes) can transmit it to their children. The genes that change are MEN1 (menin 1) and RET (ret proto-oncogene).

Both men and women are at equal risk. Also, people of any geographical or racial group can develop MEN.

What Are the Symptoms of Multiple Endocrine Neoplasia (MEN)?

Symptoms in multiple endocrine neoplasia occur due to an increase in the hormone levels that are released.

The following symptoms are noted in MEN type1, which include:

  • Bone pain.

  • Increased tendency to fractures.

  • Kidney stones.

  • Stomach or intestinal ulcers.

  • Fatigue.

  • Vision changes.

  • Headache.

  • Infertility.

  • Increased production of breast milk.

  • Acromegaly (occurs due to excess production of growth hormone secreted by the pituitary gland).

  • Cushing’s syndrome (occurs due to abnormally high levels of the hormone cortisol).

The following symptoms are noted in MEN type 2 that includes:

  • Neuromas are benign (harmless) growths of the nerve tissues of the lips and tongue.

  • Overgrowth of thyroid cells leads to thyroid carcinoma.

  • Thickening of the lips and eyelids.

  • The curvature of the spine.

  • Abnormalities of feet and thigh bones.

  • Loose bones.

  • Enlarged large intestine.

How Is Multiple Endocrine Neoplasia (MEN) Diagnosed?

A doctor diagnoses MEN based on physical examination and taking the medical and family history. Few tests may be ordered to get a better picture of the condition, which include:

  1. Magnetic resonance imaging (MRI) scans use magnetic fields and radio waves to provide precise details of the soft tissues of the body.
  2. Computerized tomography (CT) scans to create cross-sectional images using several X-rays and computer processing techniques.
  3. Positron emission tomography (PET) scan uses tiny particles called positrons to create images of the body tissues. Body images are obtained by detection of radiation from the emission of positron particles.
  4. Nuclear medicine scans use radioactive substances for the diagnosis and treatment of diseases.
  5. Ultrasound scans of the pancreas use high-frequency sound waves to create images of the body parts.

Genetic testing helps determine if a person has MEN. Children of the affected person are at a higher risk of inheriting the mutation (change). Also, parents and siblings of the affected persons may be having the condition without showing noticeable symptoms.

How Is Multiple Endocrine Neoplasia (MEN) Treated?

Treatment of multiple endocrine neoplasia depends on the type and the development of the tumors. The following pattern may be considered in treating MEN.

  • Screening for tumors.

  • Surgery to remove tumors that have developed.

  • Chemotherapy.

  • Radiation therapy.

  • Genetic counseling for parents who are planning pregnancy.

The treatments for specific tumors include:

  1. Pituitary Tumors: They can be treated by surgery or medications.
  2. Hyperparathyroidism: It is treated by removal of the overactive parathyroid glands.
  3. Hypoglycemic Syndrome: A tumor of the pancreas results in the production of excess insulin (a condition called insulinoma), which leads to very low blood glucose levels that can be life-threatening. Surgery is done to remove a part of the pancreas.
  4. Neuroendocrine Tumors: These types of tumors occur in the pancreas or small intestine. Surgery, medications, or an ablation procedure are the preferred modes of treatment. The ablation procedure destroys the tumors using extreme heat or cold.
  5. Zollinger-Ellison Syndrome (ZES): ZES involves tumors that produce excess gastric acid that can lead to diarrhea and ulcers. Medications or surgery are the treatment options.
  6. Adrenal Tumors: In most cases, adrenal tumors are treated by a wait and watch approach. However, if the tumors are cancerous (harmful), minimally invasive surgery is advised to remove them.
  7. Carcinoid Tumors: These types of tumors develop in the lungs, digestive tract, and thymus gland. Radiation, chemotherapy, or hormone-based therapy is recommended for advanced cases of carcinoid tumors.

Conclusion:

Multiple endocrine neoplasia (MEN) is an inherited condition that is passed down in families. Symptoms may occur based on the gland affected and also the number of hormones circulating in the bloodstream. Early screening based on family history can help start early treatment for the tumors. Also, regular check-ups with the endocrinologist are advised post-treatment to keep a check on the regrowth of tumors. Genetic counseling is advised for families who plan on starting a family.

Frequently Asked Questions

1.

What Exactly Is the Difference Between Multiple Endocrine Neoplasia -1 and Multiple Endocrine Neoplasia -2?

Both MEN1 (multiple endocrine neoplasia) and MEN2 (multiple endocrine neoplasia)are rare hereditary illnesses affecting the endocrine system. The key distinction between them is the types of tumors they cause. MEN1 (multiple endocrine neoplasias) is linked to parathyroid, pancreatic, and pituitary gland tumors, whereas MEN2 (multiple endocrine neoplasias) is linked to medullary thyroid cancer and pheochromocytomas.

2.

What Is the Lifespan of People With Multiple Endocrine Neoplasia?

The longevity of patients with multiple endocrine neoplasia (MEN) is determined by various factors, including the multiple endocrine neoplasia subtype, severity of symptoms, and therapy success. People with multiple endocrine neoplasia can live a normal life if they are properly managed. Complications from multiple endocrine neoplasia, on the other hand, can reduce life expectancy.

3.

How Are Multiple Endocrine Neoplasia -1 Identified?

MEN1((multiple endocrine neoplasias) is discovered using a combination of clinical and genetic testing. Typically, doctors will perform a physical exam and request blood tests to detect hormone levels. Tumors can also be detected through imaging tests such as CT scans and MRIs. By finding mutations in the MEN1 (multiple endocrine neoplasias) gene, genetic testing can confirm the diagnosis.

4.

What Is the Most Common Type of Endocrine Tumor?

The most frequent type of endocrine tumor is an adenoma, which is a benign development. Adenomas can develop in the pituitary, thyroid, and adrenal glands, among other endocrine glands. Although most adenomas are benign, some can progress to cancer. Therefore, early detection and treatment are important.

5.

What Are the Three P’s of Multiple Endocrine Neoplasia?

Multiple endocrine neoplasia (MEN) is defined in three P's: Primary hyperparathyroidism is characterized by parathyroid gland hyperactivity, resulting in excessive parathyroid hormone (PTH) release. Pituitary adenomas are noncancerous tumors of the pituitary gland that can cause hormonal abnormalities. Pancreatic islet cell tumors: tumors that form in pancreatic cells that produce hormones like insulin and glucagon, resulting in abnormal blood sugar levels and other hormonal abnormalities.

6.

What Medications Are Used to Treat Multiple Endocrine Neoplasia?

The therapy of multiple endocrine neoplasia (MEN) depends on the type and degree of the tumors involved. Medications may be used to manage symptoms or slow tumor growth, and surgery may be necessary to remove tumors that are causing problems. Somatostatin analogs, dopamine agonists, and bisphosphonates are some of the most commonly used medications to treat MEN (multiple endocrine neoplasia).

7.

What Are the Complications of Multiple Endocrine Neoplasia?

Multiple endocrine Neoplasia (MEN) can cause hormone abnormalities, bone loss, and an increased risk of cancer. Endocrine gland tumors can cause overproduction or underproduction of hormones, while primary hyperparathyroidism can cause bone weakness. Because certain forms of MEN (multiple endocrine neoplasia) can raise the chance of getting cancer, regular monitoring and screening are essential for detecting cancer early.

8.

What Organs Are Impacted by Multiple Endocrine Neoplasia?

Multiple endocrine neoplasia (MEN) affects many endocrine organs. The organs affected vary according to the type of MEN (multiple endocrine neoplasia); however, the parathyroid glands, pancreas, pituitary gland, thyroid gland, and adrenal glands are frequently involved. MEN (multiple endocrine neoplasia) can result in the formation of many forms of tumors in these organs, resulting in hormonal abnormalities and other health issues. Regular screening and monitoring are critical for the early discovery and management of MEN (multiple endocrine neoplasia).

9.

Is There a Blood Test for Multiple Endocrine Neoplasia - 1?

Yes, there is a MEN1 (multiple endocrine neoplasia) blood test that may assess the levels of hormones and other chemicals in the blood that are commonly altered by the disorder, such as parathyroid hormone, insulin-like growth factor 1 (IGF-1), and gastrin. However, in addition to blood tests, a diagnosis of MEN1 (multiple endocrine neoplasia) usually necessitates a combination of clinical evaluation, imaging exams, and genetic testing. The tests used to diagnose MEN1 (multiple endocrine neoplasia) may differ depending on the individual's symptoms and medical history.

10.

What Is Multiple Endocrine Tumour Types-1?

MEN1 (multiple endocrine neoplasia) is a rare genetic condition characterized by the development of several tumors in the endocrine glands, including the parathyroid, pancreas, and pituitary gland. These tumors are usually not malignant, but they can cause serious health issues by overproducing hormones and interfering with regular biological activities. Mutations in the MEN1 (multiple endocrine neoplasia) gene cause MEN1 (multiple endocrine neoplasia) and can be inherited in an autosomal dominant form.

11.

Is Multiple Endocrine Neoplasia -1 Is an Autoimmune Disease?

MEN1 (multiple endocrine neoplasia) is not an autoimmune disorder. It is a hereditary condition caused by mutations in the MEN1 (multiple endocrine neoplasia) genes that result in the development of numerous endocrine gland tumors. Autoimmune disorders arise when the immune system incorrectly attacks healthy tissues in the body, whereas MEN1 (multiple endocrine neoplasia) is caused by a genetic mutation that impairs the body's capacity to regulate the growth and function of specific glands.

12.

What Is Multiple Endocrine Neoplasia -1 Cushing’s Syndrome?

MEN1 (multiple endocrine neoplasia) Cushing's syndrome is a disorder that can affect people who have multiple endocrine neoplasia type 1 (MEN1). It is distinguished by the formation of tumors in the pituitary and adrenal glands that overproduce cortisol, a hormone that aids in the regulation of the body's response to stress. Cortisol overabundance can result in a number of symptoms, including weight gain, high blood pressure, and glucose intolerance.

13.

What Is the Location of Gastrinoma Multiple Endocrine Neoplasia -1?

Gastrinoma is most typically found in the duodenum, the first section of the small intestine, in multiple endocrine neoplasia 1 (MEN1). These tumors, however, can also develop in other regions of the digestive system, such as the pancreas and stomach.

14.

What Gene Is Mutated in Multiple Endocrine Neoplasia -1?

The multiple endocrine neoplasia (MEN1) gene, which is found on chromosome 11, is altered in multiple endocrine neoplasia-1 (MEN1). Mutations in this gene cause tumors in the endocrine glands, such as the parathyroid, pancreas, and pituitary gland.

15.

What Are the Components of Multiple Endocrine Neoplasia -1?

MEN1 (multiple endocrine neoplasia), or several endocrine neoplasia types 1, is characterized by the development of tumors in several endocrine glands, including the parathyroid, pancreas, and pituitary gland. Mutations in the MEN1 (multiple endocrine neoplasia) gene cause MEN1 (multiple endocrine neoplasia), which affects the body's capacity to control cell growth and division in these glands.
Dr. Thiyagarajan. T
Dr. Thiyagarajan. T

Diabetology

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multiple endocrine neoplasia
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