Endocrine Gland Neoplasm

What is endocrine gland neoplasm?

Endocrine gland neoplasm, or multiple endocrine neoplasia (MEN), is a rare genetic condition. The condition occurs when multiple tumors and/or cancer affect certain glands or tissues that are located in the endocrine system.

The organs and glands in the endocrine system that produce and release hormones are:

  • Hypothalamus
  • Pituitary gland
  • Parathyroid glands
  • Thyroid
  • Testes
  • Ovaries
  • Adrenal glands
  • Pineal gland
  • Pancreas

 

There are two main types of endocrine gland neoplasm: multiple endocrine neoplasia type 1 and multiple endocrine neoplasia type 2 (MEN-2). The first condition is when multiple tumors affect different parts of the endocrine system.

MEN-2 is a genetic polyglandular (multiple glands) cancer syndrome. There is an increased risk of developing other tumors that affect other glands within the endocrine system. Individuals with MEN-2 will develop medullary thyroid cancer, also known as carcinoma.

If you are worried that you may be at a higher risk for developing endocrine gland neoplasm, or you start to notice any symptoms, speak with a healthcare provider to learn your best options.

Who is affected by endocrine gland neoplasm?

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Men and women are affected equally by endocrine gland neoplasm. The onset for MEN-1 can vary between individuals. Diagnoses can occur as young as eight years old up to adults as old as 80. It’s still considered a rare condition, with MEN-1 affecting about 1 in 30,000 individuals. MEN-2 affects roughly 1 in 35,000 people. The rate may be higher due to undiagnosed and/or misdiagnosed persons.

What are the symptoms of endocrine gland neoplasm?

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The symptoms of MEN-1 can vary based on which of the glands are affected by an overgrowth of tissue or a tumor. Most MEN-1 tumors are non-cancerous, but tissue overgrowth and tumors can result in an overproduction of hormones in the body. Although not all MEN-1 cases appear with the same symptoms, it is important to know which are connected to specific diagnoses.

 

Hyperparathyroidism

Mild hyperparathyroidism symptoms include:

  • Depression
  • Fatigue
  • Joint pain
  • Muscle weakness
  • Loss of appetite
  • Trouble concentrating

 

Symptoms of severe hyperparathyroidism include:

  • Bone pain
  • Constipation
  • Confusion and forgetfulness
  • Increased thirst
  • Frequent urination
  • Nausea and vomiting

 

Gastrinomas

Roughly 40% of adults with MEN-1 develop multiple gastrinomas. Gastrinomas tend to appear in the pancreas or in the first part of the small intestine that is connected to the stomach. Some of the symptoms can include:

  • Diarrhea
  • Abdominal pain
  • Acid reflux
  • Peptic ulcers

 

Insulinomas

Insulinoma is another tumor that commonly affects the gastroenteropancreatic tract. A benign (non-cancerous) tumor secretes insulin. The condition can lead to low blood sugar, causing:

  • Sweating
  • Shakiness
  • Confusion
  • Hunger
  • Temporary vision changes
  • Heart palpitations
  • Anxiety

 

Prolactinomas

The most common pituitary gland tumor for cases of MEN-1 is prolactinomas. They are third most common among MEN-1 tumors after parathyroid tumors and gastrinomas. Symptoms differ between men and women.

Male symptoms often include:

  • Infertility
  • Erectile dysfunction
  • Loss of sexual interest due to low levels of testosterone

 

Female symptoms often include:

  • Infertility
  • Menstrual changes (irregular periods, no periods)
  • Loss of sexual interest
  • Milky discharge from nipples (when not pregnant or breastfeeding)

 

Large prolactinomas can also cause headaches, nausea and/or vomiting, and vision changes like double vision or reduced peripheral vision.

The symptoms of MEN-2 similarly vary like individuals experience with MEN-1. Symptoms of MEN-2 will relate back to:

 

Medullary thyroid cancer (MTC)

Everyone with type 2 endocrine gland neoplasm develops medullary thyroid cancer, but staying on top of the symptoms can help you take a proactive approach to your health with your doctor. Pay attention and make an appointment immediately if you notice:

  • Shortness of breath
  • Coughing
  • Trouble swallowing
  • A lump in the front of your neck
  • Pain in the front of your neck
  • Voice changes (hoarseness)

 

Pheochromocytomas

Roughly half of people with MEN-2 develop a pheochromocytoma, which is generally a non-cancerous tumor in your adrenal gland(s). Symptoms typically occur as spells and frequently include:

  • Shakiness
  • Excessive and unexplained sweating
  • Headache
  • High blood pressure
  • Irregular heartbeat (pounding, fast)

 

Hyperparathyroidism

If you experience hyperparathyroidism, symptoms of it may include:

  • Difficulty concentrating
  • Fatigue
  • Depression
  • Joint pain
  • Bone pain
  • Muscle weakness
  • Nausea and vomiting
  • Loss of appetite
  • Increased thirst
  • Frequent urination

 

  • Constipation

What causes endocrine gland neoplasm?

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MEN-1 and MEN-2 are caused by gene mutations. They can be inherited from your biological parent or develop randomly as an embryo. There is a 50% chance that a biological parent will pass down an endocrine gland neoplasm condition.

How is endocrine gland neoplasm diagnosed?

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MEN-1 is diagnosed if you have:

  • At least two out of three endocrine tumors (parathyroid tumor, pituitary tumor, and/or a tumor in the gastroenteropancreatic tract)
  • One of the tumors and a family history of MEN-1

 

Your healthcare provider will need to diagnose one or more tumors before they can diagnose you with MEN-1. Blood tests reveal elevated levels of specific hormones that can indicate the presence of a tumor. Other testing methods are CT or MRI scans. Providers can confirm your diagnosis by doing genetic testing of the MEN1 gene.

MEN-2 is diagnosed if you have:

  • Medullary thyroid cancer and pheochromocytoma
  • Parathyroid enlargement
  • A tumor (adenoma)

 

Blood tests can determine if you have medullary thyroid cancer or other tumors. After running these tests, a healthcare provider will use CT scans or MRI scans to detect and diagnose any tumors that may be associated with MEN-2. Providers can confirm your diagnosis with genetic testing of the RET gene.

How is endocrine gland neoplasm treated?

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Treatment options depend on the affected endocrine glands and organs. As these decisions are made, it is important to consult with a team of specialists. These may include:

  • Endocrinologists
  • Surgeons
  • Oncologists
  • Pediatricians, if applicable

 

Medication to treat symptoms and counteract the effects of excess hormones is possible. A doctor may also recommend surgery to remove tumors or the entirety of the affected gland. Other scenarios are replacement hormones once an endocrine gland is removed or cancer treatment (chemotherapy or radiation) if the cancer has spread to other areas of the body.

Do not be afraid to ask your healthcare provider questions about your specific endocrine gland neoplasm treatment. Each case is unique and requires its own tailored treatment plan.

What is the outlook for endocrine gland neoplasm?

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While there is no current cure for endocrine gland neoplasm, it is highly manageable with the help of a healthcare team. Speaking to a doctor sooner rather than later will help you receive your diagnosis and know which endocrine gland(s) is affected. Early diagnosis is beneficial for knowing whether a tumor is benign or cancerous, plus the best treatment to help address if it has spread to any other part of the body.

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