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General Topics

Overview of Endocrine Neoplasia and Cancer | Endocrinology

What are endocrine neoplasia and cancer?

Endocrine neoplasia refers to growths or tumors that affect the hormone-producing endocrine system. Tumors develop in the adrenal glands, pituitary gland, parathyroid glands, or pancreas and can be cancerous or benign. The Nig Comprehensive Cancer Center‌ Endocrine Neoplasia Program is dedicated to the evaluation and treatment of the structural and hormonal symptoms of these disorders.

Our endocrine neoplasia program is the only one in Connecticut, and our dedicated team of experienced multidisciplinary specialists provides clinical services in the following areas:

  • Thyroid cancer and thyroid nodules
  • Fine injection aspiration biopsy: Thyroid and adrenal
  • Thyroid ultrasound
  • Thyroid carcinoma test: Thyrogen stimulated thyroglobulin and thyroid scan
  • Cancer and benign tumors of the adrenal glands.
  • Invasive radiology including petrous sinus sampling, adrenal vein sampling, and selective infusion of pancreatic calcium
  • Hyperparathyroidism and parathyroid tumors
  • Pituitary adenomas
  • Dynamic endocrine examination
  • Nuclear medicine scan

With specialists in a variety of diagnostic and therapeutic approaches, we work with teams of physicians from other disciplines to treat patients with endocrine neoplasia. Our medical professionals have experience in endocrine surgery, urological surgery, neurosurgery, neuro-ophthalmology, pathology, nuclear medicine, invasive radiology, hypertension, genetics, molecular biology, and endocrinology. They provide state-of-the-art patient care while conducting basic and clinical research to advance treatment options.

Types of endocrine neoplasia and cancer

Tumors can appear in any large endocrine gland, including the thyroid, parathyroid, pituitary, and adrenal glands, and the pancreas. The most common sites are:

  • Thyroid gland: Most endocrine cancers develop in the thyroid gland (a butterfly-shaped organ in the lower neck). Thyroid cancer is more common in women than in men. Statistics show that the annual rate of thyroid cancer is increasing in the United States and around the world. The good news is that most thyroid tumors (called nodules) are not cancerous.
  • Pituitary gland: A pea-sized organ connected to the brain, the pituitary gland produces hormones that affect growth and fertility. Pituitary tumors are almost always benign, but they contain more or less than one or more hormones, which can upset the balance of other glands.
  • Adrenal gland: The two adrenal glands that live above the kidneys produce hormones that regulate metabolism (cortisol), stress response (adrenaline), blood pressure (aldosterone), and certain sexual characteristics (androgens).
  • Pancreas: Although the pancreas plays an active role in the digestive system, it is also a source of important hormones, including insulin. Rare tumors produce too much insulin or other related hormones, which can affect blood sugar levels.

Although some cases are inherited, the cause of most endocrine cancers is generally unclear.

Symptoms of endocrine neoplasia and cancer

Some patients with thyroid tumors notice a lump in the neck. For others, and for other endocrine tumors, the general rules do not apply. Some tumors cause severe hormonal changes or discomfort, while other tumors do not have any symptoms.

So when does a tumor have symptoms? You basically have symptoms if it doesn’t work (makes extra hormones) but is active (doesn’t make them). For example, an adrenal tumor that produces excess testosterone can cause a patient to develop certain male characteristics, such as facial hair. Symptoms also appear as the tumor grows.

A large tumor destroys part of the gland, causing a lack of hormones. It also affects nearby structures. For example, a large pituitary tumor can focus on the nerve that runs between the eyes and the brain, causing vision changes. When endocrine tumors have no symptoms, doctors may randomly notice them and evaluate the patient for another reason.

What are the genes associated with multiple endocrine neoplasias?

Mutations in the MEN1, RET, and CDKN1B genes cause multiple endocrine neoplasms. Mutations in the MEN1 gene cause type 1 multiple endocrine neoplasias. This gene provides instructions for the production of a protein called melanin. Menin acts as a tumor suppressor, which means that it generally prevents cells from growing and dividing too quickly or uncontrollably.

Although the exact function of the meninges is unknown, it is involved in cellular functions such as DNA copying and repair and regulation of the activity of other genes. When mutations inactivate two copies of the MEN1 gene, the meninges are no longer available to control cell growth and division. Loss of functional meninges allows cells to divide more frequently, leading to tumor characterization of multiple endocrine neoplasia type 1. It is not clear why these tumors affect endocrine tissues.

Mutations in the RET gene can cause type 2 multiple endocrine neoplasias. This gene provides instructions for the production of a protein involved in cell signaling. The RET protein stimulates chemical reactions that direct cells to respond to their environment, for example by dividing or maturing. Mutations in the RET gene over-activate the protein’s signaling function, which stimulates cell growth and division in the absence of signals external to the cell. This unproven cell division can lead to the formation of tumors in the endocrine glands and other tissues.

Mutations in the CDKN1B gene cause type 4 multiple endocrine neoplasias. This gene provides instructions for the production of a protein called p27. Like the meaning protein, p27 is a tumor suppressor that helps regulate cell growth and division. Mutations in the CDKN1B gene reduce the number of functional p27 that allows cells to grow and divide without being analyzed. This irregular cell division leads to the development of tumors in the endocrine glands and other tissues.

Diagnosis of endocrine neoplasia and cancer

Doctors can perform several tests to check for a suspected endocrine tumor:

  • A medical history and physical exam to assess for physical or behavioral changes related to hormone function
  • Lab tests to check for abnormal hormone levels in the blood or urine
  • Imaging studies (CT scan, MRI, or ultrasound) to look for evidence of abnormal tissue in the gland
  • A biopsy to obtain a sample of abnormal tissue and analyze it for cancer cells

Treatment for endocrine neoplasia and cancer

For any endocrine tumor, treatment involves surgery to remove it. For people with cancer, another approach that is sometimes used is radiation therapy. Patients sometimes receive hormone therapy to balance the level of hormones in the body. Depending on the type of tumor, your doctor may prescribe other specific rules to meet your individual needs.

Categories
Procedures

Overview of Adrenalectomy | Endocrinology

What is Adrenalectomy?

There is one adrenal gland above each of your kidneys. The adrenal glands produce different hormones that help regulate metabolism, the immune system, blood pressure, blood sugar, and other basic functions.

Most adrenal gland tumors are noncancerous (benign). You may need surgery to remove the adrenal gland if the lump is producing excess hormones or is large (more than 2 inches or 4 to 5 centimetres). You may also need surgery if the lump is precancerous (malignant) or suspected to be precancerous. This surgery is called adrenalectomy.  You might also need adrenalectomy to remove cancer that has spread (metastasized) from additional location, such as the kidney or lung.

If both adrenal glands are removed, you will need to take supplemental hormone medications. If only one gland is detached, the other will take over full function without the need for ongoing medications.

Types of adrenalectomy

Surgeons may perform adrenalectomy through minimally invasive (laparoscopic) surgery, traditional open surgery, or the use of cryoablation. The procedure they commend depends on the extent and type of tumor or the condition affecting your adrenal gland.

Minimally invasive surgery:  Endocrine surgeons are often capable to use minimally invasive (laparoscopic) surgery for tumors of the adrenal gland because the gland is relatively small. Laparoscopic surgery has many benefits, including smaller scarring, less pain and a shorter recovery period than traditional open surgery.

An alternative approach to laparoscopic surgery is endoscopic posterior adrenalectomy (PRA), in which surgeons make small incisions in your back. Surgeons sometimes use the robotic da Vinci surgical system to perform laparoscopic adrenalectomy.

  • Open surgery: Doctors usually reserve open surgery for large or cancerous (malignant) tumors. They perform open surgery using incisions and traditional tools.
  • Cryoablation: Additionally, doctors may use cryoablation to treat adrenal gland tumors. This procedure uses a CT scan to guide the insertion of a probe that freezes and destroys adrenal tumors. Interventional radiologists may use cryoablation as a treatment option for small tumors that have spread to the adrenal gland (metastasis), especially when surgery involves high risk.

How is adrenalectomy done?

The surgeon will work in one of two ways:

  • Open adrenalectomy: The surgeon may use open surgery if the lump is large or it may be cancerous. The surgeon makes a large open incision (cut) in the abdomen to remove the gland (s).
  • Laparoscopic adrenalectomy: Most commonly, the surgeon makes a few small incisions and performs a minimally invasive procedure. This method uses a laparoscope, which is a thin tube equipped with a tiny video camera so that the surgeon can see what is inside the body. Robotic surgery uses the same incisions but uses wrist tools instead of solid ones. Whether the procedure is laparoscopic or robotic, the procedure can be performed either by placing incisions on the back (posterior approach) or on the side (lateral approach).

Both procedures are equally successful, with the posterior approach providing the advantages of not entering the abdomen. The back method is also preferred in patients with a previous history of upper abdominal incisions and two-sided tumors. Due to the small working area with the posterior approach, only tumors smaller than 6 cm are approached through the back.

Does adrenalectomy remove one gland or both?

The surgeon may remove the tumor, only one gland, or both. The surgery that removes one gland is a unilateral removal of the adrenal gland. Two-sided adrenalectomy removes both glands. People with excess cortisol (Cushing’s syndrome may take a hydrocortisone supplement for about a year after surgery until the remaining gland begins producing enough hormone on its own. For other patients, testing is done after surgery to determine if the patient needs to be on a steroid substitute).

After the adrenalectomy procedure

The hospital stay after surgery is usually 4 to 5 days. The patient can return to his normal activities as soon as he feels ready. Patients are advised to avoid strenuous exercise for 6-8 weeks after surgery.

Benefits of adrenalectomy

The advantages of adrenalectomy treatment are:

  • Smaller scars
  • Less risk of hernia
  • Pain relief after the operation
  • Shortest hospital stay and recovery time

Risks of adrenalectomy

The risks associated with adrenalectomy are:

  • Hormonal imbalance is a potential risk, leading to complex health problems such as slow healing, blood pressure disorders or metabolic disorders.

Other potential risks include

  • Bleeding
  • Bowel disorders
  • Blood clots in the lungs
  • Infections
  • Pain
  • Scarring, or damage to the pancreas.

Limitations

  • Damage to neighbouring organs
  • Hernia
Categories
Disease

What Are Your Adrenal Glands? | Endocrinology

Overview of adrenal glands

The adrenal glands are small glands that sit above the kidneys in the upper abdomen. They produce and release several hormones in the body.

A range of medical conditions can affect the adrenal glands. These include Addison’s disease, Cushing’s syndrome, and adrenal cancer, as well as high blood pressure due to the overproduction of aldosterone.

What are the symptoms of adrenal glands?

People with adrenal gland tumours may experience the following symptoms or signs. Sometimes people with adrenal gland tumours do not have any of these changes or the symptom may be caused by another medical condition other than a tumour.

  • Hypertension
  • Low potassium level
  • Heart palpitations
  • Nervous
  • Feelings of anxiety or panic
  • Headache
  • Excessive sweating
  • Diabetes
  • Abdominal pain
  • Unexplained weight gain or loss
  • Weakness
  • Abdominal stretch marks
  • Excessive hair growth
  • Changes in the genitals
  • Abnormal pimples
  • Change in libido (sex drive)

Also, pheochromocytoma can cause a dangerous increase in hormones that regulate blood pressure and the body’s response to stress. Hormone increases can cause blood pressure to rise too quickly, increasing the risk of heart attack, stroke, bleeding, or sudden death.

If you are concerned about any changes you may experience, speak with your doctor. Among other things, your doctor may ask you how often and how often you experience symptoms. It helps find the cause of the problem called the diagnosis.

If the tumour is diagnosed, relief of symptoms is an important part of medical care and treatment. This is also known as symptom management, palliative care, or supportive care.

Risk factors of adrenal glands

Any risk factor that makes a person more likely to develop a tumour. Although risk factors often influence tumour development, most do not directly cause it. Some people with multiple risk factors never develop a tumour, while others have no risk factors. However, knowing your risk factors and talking to your doctor about them can help you make more informed decisions about your lifestyle and health care.

The cause of most cancerous tumors of the adrenal glands is unknown. However, people with certain inherited conditions are at increased risk of developing adrenal gland tumours. Hereditary conditions that increase the risk of adrenal gland tumours:

  • Multiple endocrine neoplasia type 2 (MEN2)
  • Li-Fraumeni syndrome
  • Von Hippel-Lindau syndrome
  • Neurofibromatosis type 1
  • Carney Complex

Researchers are still struggling to gather enough evidence before reaching final conclusions about what causes this type of tumor and what people can do to reduce their personal risk.

Diagnosis of adrenal glands

Doctors use many tests to find or diagnose, a tumour. They also do tests to learn if a tumour is cancerous and if it has spread to another part of the body from where it started. If this happens, it is called metastasis. Some tests may also determine which treatments may be the most effective.

To diagnose an adrenal gland tumour, blood and urine tests look for the presence of certain substances to help determine whether the tumour is functional or nonfunctional. A computed tomography (CT or CAT) scan or a magnetic resonance imaging (MRI) scan (see below) may be useful in making a diagnosis and evaluating whether an adrenal gland tumour is cancerous. Imaging tests may also be used to find out whether a cancerous tumour has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments could work best.

This list describes options for diagnosing this type of tumour, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • The type of tumour suspected
  • Your signs and symptoms
  • Your age and medical condition
  • The results of earlier medical tests

In addition to a thorough physical examination, the following tests may be used to diagnose an adrenal gland tumor:

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. If the doctor suspects that cancer has spread to the adrenal gland from another part of the body, a biopsy may be done to find out where cancer began, which can help the doctor plan treatment.  If this is necessary a narrow, hollow needle is used to collect the tissue. This is called a fine-needle biopsy or fine-needle aspiration.

The biopsy is performed by a radiologist who uses specialized imaging procedures, such as CT scans, to guide the needle directly into the tumour. A pathologist then analyzes the sample(s) removed during the biopsy. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

CT or CAT scan. A CT scan creates a 3-dimensional picture of the inside of the body using x-rays taken from different angles A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumours. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye is often injected into a patient’s vein through a peripheral intravenous (IV) line. This line is a short, plastic tube inserted into the vein that allows the health care team to give medication or fluids.

MRI. An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure the tumour’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow.