What is primary hyperparathyroidism?

Primary hyperparathyroidism is a condition in which your blood calcium levels are elevated, usually because of an enlarged parathyroid gland(s). Parathyroid glands control your calcium, like a thermostat controls the temperature in your house. If your house is 90 °F, then the thermostat should turn "off" the heater. If the heater is still "on," then your thermostat is broken.

Similarly, your parathyroid glands control the calcium in your blood. If your serum calcium is high, then your parathyroid glands should be "turned off" (i.e. your parathyroid hormone (PTH) should be low). If your serum calcium is high and your parathyroid glands are making lots of PTH, then one or more parathyroid glands are not working correctly.

What are the symptoms of hyperparathyroidism?

Hyperparathyroidism can cause a host of different symptoms. The most common signs and symptoms we see patients for are weak bones (osteopenia or osteoporosis) or kidney stones. Some symptoms of hyperparathyroidism are more subtle and can include changes in your kidney function, worsening fatigue, short term memory loss, muscle or bone pain, depression, anxiety, abdominal cramps or frequent urination. Very high blood calcium levels can cause seizures and heart problems.

What is a minimally invasive parathyroidectomy?

The phrase "minimally invasive parathyroidectomy" is used to encompass a range of different procedures that strive to minimize the length of the surgical incision; therefore, facilitating a quicker recovery and better cosmesis. For decades we made an incision to look at all four parathyroid glands. Nowadays, we use imaging studies (like ultrasound) to focus our operation and remove just the one enlarge parathyroid gland that is causing the problem. This focused; image directed parathyroidectomy is frequently referred to as a minimally invasive parathyroidectomy. Typically the incision is around 2.5 cm (about 1 inch).

What is parathyroid cancer?

Parathyroid cancer is very rare. Most people with primary hyperparathyroidism have a parathyroid adenoma which is a benign, enlarged parathyroid gland. Patients with parathyroid cancer typically have a very high serum calcium level (greater than 14 mg/dL).

Why do I need to get localizations studies such as an ultrasound, sestamibi scan or CT scan?

Almost all people have four parathyroid glands. Most patients with primary hyperparathyroidism have only one gland that his enlarged and malfunctioning. Localization studies will allow your surgeon to identify which gland is the likely culprit and therefore minimize the extent of surgery to quicken your recovery and improve cosmesis.

My localization studies don't show an enlarged parathyroid adenoma, does that mean I don't have hyperparathyroidism? Can I still have surgery?

Hyperparathyroidism is based on the finding of elevated serum calcium with an elevated parathyroid hormone (PTH). Localization studies help surgeons determine the type of surgery to perform, they do not determine if you have hyperparathyroidism. If you have negative localization studies, you can still have surgery, but you may require further imaging or a bilateral (4-gland) exploration.

What if I had a previous unsuccessful parathyroid surgery?

About 5% of patients will develop persistent or recurrent hyperparathyroidism after a previous parathyroid surgery. Persistent hyperparathyroidism means that your calcium never improved after your previous surgery. Recurrent hyperparathyroidism means that your calcium returned to normal for at least 6 months and then became elevated again. This usually means that you have more than one enlarged parathyroid gland. We are experts in the management of persistent and recurrent hyperparathyroidism and are happy to accept patients with previous failed explorations or recurrent hyperparathyroidism.

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I have a thyroid nodule. Does that mean I have cancer?

Thyroid nodules are very common (about 50%-70% of the population have them). Fortunately the vast majority are benign (95%) and non-bothersome. To evaluate whether your nodule is cancer we frequently perform a Fine-Needle Aspiration (FNA). In general, FNA's are well tolerated and very accurate.

My FNA came back as a follicular lesion or indeterminate. What does that mean?

When we FNA a nodule there are essentially three results we can get. The nodule may be 1) benign, 2) malignant (cancer) or 3) indeterminate. Indeterminate findings can occur for a variety of reasons, but the most common is follicular tumors. Unfortunately, often we cannot tell if a follicular tumor is malignant or benign on biopsy. The overall risk of cancer is usually around 20% in these cases. Historically, the recommended treatment for these findings is surgical removal to allow for diagnosis; however, recently the option of gene expression profiling has emerged as a potential avenue.

What is Afirma© "gene expression" testing?

When you have a thyroid nodule with an indeterminate FNA, then the most common recommendation is for surgical removal to a facilitate diagnosis. Gene expression testing is a new technology that provides additional information on the nodule based on its DNA and RNA fingerprint. This allows us to better quantitate the risk that the nodule is cancer.

I have a multinodular goiter. What does that mean?

Goiter simply means that you have a big thyroid. Multinodular means that your goiter is composed of many thyroid nodules. Most multinodular goiters are benign, but when they get big enough they may cause symptoms. The most frequent symptoms are problems breathing (especially when lying flat), difficulty swallowing, or hoarseness.

What is minimally invasive surgery?

Minimally invasive surgery for the thyroid strives to minimize the size of the incision to facilitate faster healing and improved cosmesis. Some thyroid surgeries can be performed through an incision that is approximately 3 cm (1-inch).

I have Graves' disease. Is surgery right for me?

Graves' disease is an autoimmune disease where the body makes antibodies that stimulate production of thyroid hormone. There are three treatment options for patients with Graves' disease including taking a medication (such as methimazole), radioactive iodine or surgery. All these treatments have benefits and drawbacks, but surgery is an option for patients with Graves' disease.

I have a newly diagnosed thyroid cancer. What are my treatment options?

There are four types of thyroid cancer, but in general most people diagnosed with thyroid cancer have papillary thyroid cancer or follicular thyroid cancer. These are called differentiated thyroid cancer. The treatment of differentiated thyroid cancer involves three parts.

The corner stone of treatment for differentiated thyroid cancer is surgery with removal of the entire thyroid. In addition to surgery, some patients will require radioactive iodine following surgery to lower the chance of the thyroid cancer returning. Finally, after surgery you must take a thyroid supplement (ie synthetic thyroid hormone) to keep your brain from stimulating growth of any thyroid cancer cells. This is called thyroid suppression therapy.

I have a newly diagnosed papillary thyroid cancer. What is my prognosis?

Overall patients with papillary thyroid cancer have a very good prognosis. Thyroid cancer can recur in the neck lymph nodes even years after your operation. These lymph node recurrences may require an additional surgery to remove them. This is called a neck dissection. It is important that if you have a thyroid cancer that you get regular follow up with your physicians to monitor for a recurrence which usually includes a neck ultrasound and blood tests.

What is robotic thyroid surgery?

Robot assisted thyroid surgery is a branch of remote access surgery which allows the surgeon to move the incision out of the neck to the arm pit or hair line. Although this improves cosmesis, often there are increased risks due to the amount of dissection necessary to reach the thyroid. Because of these risks, the FDA suspended approval of robotic thyroid surgery in 2011.

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What is the adrenal gland?

You have two adrenal glands. They rest in the back of your abdomen just above your kidneys. The adrenal glands are responsible for producing several hormones that help regulate many things in your body. The outside layer of you adrenal glands (called the cortex) makes three main hormones:

  1. cortisol – which regulates your response to stress
  2. aldosterone – which helps control you blood pressure
  3. androgens and estrogens – which control your reproductive organs

The inside of your adrenal glands (called the medulla) makes catecholeamines (epinephrine, norepinephrine and dopamine). These hormones are frequently referred to as "adrenaline" and mediate your "flight or fight" response. Tumors of the adrenal gland frequently produce one of the above hormones.

My doctor told me I need to have one adrenal gland removed. Can I survive with only one adrenal gland?

Yes. Only one (and sometimes even a portion of one) adrenal gland is necessary to function normally.

What is a pheochromocytoma?

A pheochromocytoma is a tumor of the adrenal gland that produces catecholamines (too much adrenaline). Historically patients with pheochromocytomas would have symptoms of headaches, sweating and heart palpitations, but in modern times, these tumors are frequently found incidentally on a CT scan done for something else. Many times patients are totally asymptomatic.

What is an adrenal incidentaloma?

An adrenal incidentaloma is an adrenal tumor that is found during a CT, MRI or ultrasound being done for an unrelated reason. Adrenal incidentalomas occur in about 1-7% of patients and are more common as we get older. Most adrenal incidentalomas are benign, but they must be evaluated by an experience physician to see if they are secreting hormones or potentially a cancer.

What is Cushing's syndrome?

Cushing's syndrome means you have too much of the hormone cortisol in your blood. Cortisol is important in your response to stress and plays a role regulating your blood sugars, immune response, mood and weight. Patients with Cushing's syndrome frequently present with weight gain, easy bruising, high blood pressure, diabetes, weakness, mood changes and changes in their appearance such a "moon face" or "buffalo hump." There are many causes of Cushing's syndrome, but sometimes it is caused by a tumor in the adrenal gland. Some patients are noted to have an elevated cortisol level without symptoms of Cushing's syndrome. We call this sub-clinical Cushing's syndrome.

What is an aldosteronoma?

Aldosterone is a hormone produced by your adrenal glands that helps regulate your blood pressure. Small tumors of the adrenal gland can produce too much of this hormone leading to high blood pressure. These tumors are called aldosteronomas.

What is adrenal cancer? My doctor told me I have an adrenal tumor. Does this mean I have a cancer?

Adrenal cortical cancers are fairly uncommon. About 10% of incidentally discovered adrenal tumors are adrenal cancer. Most incidentally discovered adrenal tumors are not cancer, but they still may produce hormones that affect your blood pressure, mood, weight, etc. Other cancers can spread to the adrenal glands. We call these metastases. It is unusual for an adrenal tumor to be the first finding of a cancer from some other location.

How are adrenal tumors removed?

Adrenal tumors are usually removed with a minimally invasive surgery called a laparoscopic adrenalectomy. When you remove an adrenal tumor you remove the associated adrenal gland with it. Your body does fine using your other adrenal gland to secrete the hormones necessary for your daily living.

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Endocrine Tumors of the Pancreas

My doctor told me I have a pancreatic neuroendocrine tumor (PNET). Does this mean I have pancreatic cancer?

Pancreatic neuroendocrine tumors (PNETs) are uncommon tumors that arise from the islet cells of the pancreas. These tumors are different from pancreatic adenocarcinoma which arises from the ductal cells of the pancreas and is much more common. In general, PNETs tend to be slow growing have a much better prognosis than pancreatic adenocarcinoma. Often it is difficult to tell if a PNET is benign or malignant even after surgical removal.

I have a pancreatic neuroendocrine tumor (PNET). Does that mean I need a Whipple procedure?

Pancreatic neuroendocrine tumors (PNETs) are uncommon tumors that differ from the more commonly seen pancreatic adenocarcinoma. PNETs tend to be slow growing and less likely to be a malignant, so often we remove them with a minimally invasive surgery called a laparoscopic enucleation. Some PNETs do require formal pancreatic resection. The decision on which operation you need depends on many factors including the type of tumor, its location in the pancreas and how close it sits to important structures such as the pancreatic duct.

I have a non-functional neuroendocrine tumor of the pancreas. What does that mean?

Pancreatic neuroendocrine tumors (PNETs) are frequently divided into two groups. Those that produce hormones that cause symptoms (such as insulinomas, glucagonomas, VIPoms, etc) are called functional PNETs and those that do not produce hormones that cause symptoms are called non-functional PNETs. About half of PNETs are non-functional. These are frequently found incidentally on abdominal imaging done for some other reason.

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Familial (Genetic) Endocrine Disorders

What is Multiple Endocrine Neoplasia?

Specific genetic abnormalities can lead to the development of endocrine tumors. One of the most common of these genetic abnormalities is Multiple Endocrine Neoplasia (MEN). MEN is broken into different groups depending on the specific mutation. Patients with MEN type 1 (MEN 1) are prone to get tumors of their pituitary gland, parathyroids and pancreas. Patients with MEN type 2 (MEN 2) are further divided into two sub-groups MEN 2A and MEN 2B.

Patients with MEN 2A get tumors in their thyroid (medullary thyroid cancer), parathyroid and adrenal glands (pheochromocytomas). Patients with MEN 2B get tumors in their thyroid (medullary thyroid cancer), adrenal glands (pheochromocytomas) and often have small benign tumors on their lips, tongue and eyelids. Often patients with MEN 2B also have a “marfanoid” body habitus, which means they are tall and thin with long hands.

MEN syndromes are autosomal dominant which means that they are past down from a single parent and that each offspring has a 50% chance of getting the disease.

My mom/dad/sister/brother had papillary thyroid cancer and so do I. Does this mean we have a familial predisposition?

Family history is a risk factor for papillary thyroid cancer, but represents the minority of cases. The risk of a familial papillary thyroid cancer increases with the more family members that are affected. When a single additional family member has papillary thyroid cancer, then risk of a familial syndrome is about 40%. When 3 or more family members are affected then the risk increases to 99%. Some families have rare syndromes that predispose them to colon and thyroid cancers.

My mom/dad/sister/brother had hyperparathyroidism and so do I. What does that mean?

Family history is a risk factor for primary hyperparathyroidism. This may put you at increased risk of having more than one enlarged parathyroid gland (multiple gland disease). Some families have a condition call familial hypocalciuric hypercalcaemia where the calcium receptor in the parathyroid and kidney does not work properly. These patients do no benefit from parathyroidectomy.

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