We all experience minor changes and discomforts in our bodies from time to time, right? Maybe it's a small lump in the neck, or it could be something like feeling tired for no reason, or feeling thirsty all the time. Although most of these things are not something to worry about, in rare cases, they can be the main symptoms of a medical condition. Today we are going to talk about a type of cancer that is a little rare, but it is very important for us all to be aware of. That is cancer that develops in the parathyroid glands, medically called parathyroid cancer , or `(Parathyroid Carcinoma)`.
What is Parathyroid Cancer?
Simply put, parathyroid cancer is a very rare type of cancer that develops in the tissues of your parathyroid glands. Your parathyroid glands are usually located behind the butterfly-shaped thyroid gland in the front of your neck.
Parathyroid cancer is not classified into four stages (the ``four-stage naming system``) like other types of cancer. Instead, doctors divide the cancer into three main types:
- Localized: This means that the cancer is only in your parathyroid gland. Sometimes it may have spread a little to the surrounding tissue. This means that the cancer is still confined to one area.
- Metastatic: This is when the cancer has spread to other parts of your body, such as the lymph nodes, liver, lungs, bones, or pancreas. Between 10% and 30% of people with parathyroid cancer have the cancer spread (metastatic) at the time of diagnosis. The most common sites are the lungs, bones, or liver.
- Recurrent: This is when the cancer comes back after initial treatment – usually after the cancerous gland has been surgically removed. More than half of people with parathyroid cancer will have this type of cancer. The cancer usually comes back within two to five years of the first surgery. However, it can sometimes come back as late as 20 years.
What are these parathyroid glands?
Most of us have four pea-sized parathyroid glands. As mentioned earlier, these are located behind the thyroid gland. Like the thyroid gland, the parathyroid glands are part of your endocrine system. Sometimes, these parathyroid glands can be located along your esophagus or even in your chest. These are called ectopic parathyroid glands, which means they are not where they should be.
The main function of your parathyroid glands is to control the amount of calcium in your blood by producing parathyroid hormone (PTH) . Sometimes, one or more of your parathyroid glands start to produce too much of this parathyroid hormone (PTH). This condition is called Primary Hyperparathyroidism (pHPT) . When too much PTH is produced, the calcium level in your blood increases. This condition is called hypercalcemia .
What is the difference between parathyroid cancer and parathyroid adenoma?
A parathyroid adenoma is a benign, noncancerous growth in the parathyroid gland. However, parathyroid cancer is the development of malignant, noncancerous cells in your parathyroid tissue. Of the diseases associated with the parathyroid glands, parathyroid adenoma is much more common than parathyroid cancer.
But here's the important thing: In both these conditions, parathyroid cancer and parathyroid adenoma, your parathyroid gland can become overactive and produce too much parathyroid hormone (PTH). Then, as mentioned earlier, hypercalcemia occurs. This hypercalcemia can be harmful to your body and health. Therefore, the most common treatment for parathyroid cancer, as well as parathyroid adenomas, is to surgically remove the overactive parathyroid gland.
Even for doctors, it can be difficult to immediately tell whether it is a parathyroid adenoma or a parathyroid carcinoma. Because the cells of both can look similar, after the overactive parathyroid gland is surgically removed, further tests are needed to confirm whether it is an adenoma or a carcinoma.
What is hypercalcemia? How is it related to parathyroid cancer?
Hypercalcemia is when your blood calcium levels are higher than normal. People with parathyroid cancer often develop hypercalcemia. This is because the cancer causes your parathyroid gland to become overactive and produce too much parathyroid hormone (PTH). Parathyroid hormone (PTH) regulates the amount of calcium in your blood. So, if you have too much parathyroid hormone (PTH), your blood calcium levels will increase.
Hypercalcemia can be harmful to your health and body. Therefore, it is very important to treat the hypercalcemia caused by the parathyroid cancer as well as the cancer itself.
Are there different types of parathyroid cancer?
Yes, there are two main types of parathyroid cancer.
1. The most common type: In this case, cancer cells cause your parathyroid gland to produce too much parathyroid hormone (PTH) and release it into the blood. This causes the calcium level in your blood to rise.
2. Non-functioning parathyroid carcinoma: This is also called ``non-secreting parathyroid carcinoma''. This is different from the other types. Because, people with this type of cancer do not have elevated levels of parathyroid hormone (PTH) or calcium in their blood. This type is very rare, occurring in less than 10% of parathyroid cancer patients.
Once parathyroid cancer is diagnosed, further tests are done to determine whether it is localized , metastatic , or recurrent . As mentioned earlier, the four-stage classification used for other cancers is not used for this.
Who is more likely to develop parathyroid cancer?
This cancer affects women and men equally. It is usually seen in people over the age of 30 .
How common is this disease?
Parathyroid cancer is a very rare disease. Just think, it only accounts for 0.005% of all cancers. Also, parathyroid cancer accounts for between 0.5% and 1% of all parathyroid gland-related diseases. So, this is really rare.
What are the causes of Parathyroid Cancer?
In fact, no specific cause has yet been found for the development of parathyroid cancer.
However, the following rare genetic (i.e., inherited from family) conditions are considered risk factors for this cancer:
- Multiple endocrine neoplasia type I (MEN1)
- Familial isolated hyperparathyroidism (FIHP)
- Hyperparathyroidism-jaw tumor syndrome
Additionally, if you have had radiation therapy to your head or neck in the past, it can also increase your risk of developing parathyroid cancer.
What are the symptoms of Parathyroid Cancer?
Most often, the symptoms of parathyroid cancer are the symptoms of hypercalcemia that result. Symptoms and signs of hypercalcemia may include:
- Needing to urinate more often than usual (frequent urination)
- Feeling thirstier than usual
- Nausea and vomiting
- Not feeling as hungry as usual
- Constipation
- Feeling very tired
- Feeling like you're depressed
- Forgetfulness or memory loss
- Muscle pain, weakness, and/or limping
Other possible symptoms of parathyroid cancer:
- Having a lump in the neck
- Voice changes or hoarseness
- Difficulty swallowing food
If you have one or more of these symptoms, don't be afraid to assume that it's definitely parathyroid cancer. These symptoms can also be seen in many other conditions. However, if something like this persists, it's best to see a doctor for advice.
How is Parathyroid Cancer diagnosed?
Diagnosing parathyroid cancer can sometimes be a bit challenging. One reason is that the cells of a noncancerous parathyroid adenoma and those of a parathyroid cancer can look the same.
Parathyroid cancer is often diagnosed after your abnormal, overactive parathyroid gland is surgically removed (a `parathyroidectomy`), and the tissue is further examined. Sometimes, the surgeon can detect parathyroid cancer during surgery.
What tests are done for diagnosis?
As mentioned earlier, parathyroid cancer is often diagnosed after you have been diagnosed with primary hyperparathyroidism , and the abnormal, overactive parathyroid gland is surgically removed. Sometimes, the surgeon can diagnose parathyroid cancer during the surgery.
Before your surgery to remove your hyperactive parathyroid gland, you may have tests like these:
- Blood calcium test
- Blood PTH test
- Parathyroid scan - This is usually a radioactive sestamibi scan and/or a CT scan.
If you are diagnosed with parathyroid cancer, your doctor may order imaging tests such as these to see if the cancer has spread (metastasized) to other parts of your body:
- CT scan (computerized tomography scan): A CT scan uses X-rays and a computer to create many three-dimensional (3D) images of your body.
- MRI scan (magnetic resonance imaging): An MRI scan uses a large magnet, radio waves, and a computer to make detailed images of your body. It does not use X-rays (radiation).
What are the treatments for parathyroid cancer?
The main treatment option for parathyroid cancer is surgical removal of the cancerous parathyroid gland (`en bloc resection`). If parathyroid cancer has spread (metastasized), your surgeon may also need to remove tissue around your parathyroid gland or cancerous tissue from other parts of your body.
The following surgical techniques can be used for parathyroid cancer:
- En bloc resection: In this procedure, the surgeon removes your entire parathyroid gland and the capsule around it. Sometimes, they may also remove half of the thyroid gland on the same side as the cancerous parathyroid gland, along with surrounding tissue, muscles, and nerves.
- Tumor debulking: In this procedure, the surgeon removes as much of the tumor as possible. Some tumors may not be completely removed.
- Metastasectomy: In this, the surgeon removes any cancer that has spread (metastasized) to other tissues and/or organs in your body, for example, the lungs.
Will I have chemotherapy or radiation therapy?
Chemotherapy and radiation therapy are not usually used to treat parathyroid cancer. Your doctor will decide if chemotherapy and/or radiation therapy are a suitable option for you.
Is there a cure for parathyroid cancer?
Currently, there are no specific medications for parathyroid cancer. Many people with parathyroid cancer develop symptoms of hypercalcemia. Because hypercalcemia can be harmful, your doctor may prescribe one or more of the following hypercalcemia medications:
- Cinacalcet (Cinacalcet - Sensipar®)
- Bisphosphonates
- Denosumab (Denosumab - XGEVA®)
If you have severe symptoms of hypercalcemia, your doctor may recommend that you be hospitalized immediately and begin intravenous fluids (IV) and other treatments.
Can my parathyroid cancer come back?
Yes, more than half of people diagnosed with parathyroid cancer will have a recurrence of the cancer. The cancer usually comes back within a few years – usually between two and five years. But in some cases, this can happen decades (20 years or more) after your initial diagnosis and treatment.
Can this cancer spread (metastasize) to other parts of the body?
Yes, parathyroid cancer can spread (metastasize) to other parts of your body. Between 10% and 30% of people with parathyroid cancer have the cancer already spread (metastatic) at the time of diagnosis. In these cases, the cancer most often spreads to your lungs, bones, or liver. However, it can also spread to your lymph nodes or pancreas.
What is the prognosis of parathyroid cancer?
The prognosis for parathyroid cancer depends on how complete the surgery to remove the parathyroid gland is when you are first diagnosed, as secondary treatment options such as radiation therapy and chemotherapy are limited.
For people with parathyroid cancer who have undergone an en bloc resection and have negative margins, the survival rate is as high as 90% five years after the initial surgery. The survival rate is also as high as 67% ten years after the initial surgery.
More than half of people diagnosed with parathyroid cancer and having surgery to remove the gland will experience a recurrence of the cancer. In these cases, the cancer usually comes back within two to five years of the initial surgery. However, it can come back decades later.
Can Parathyroid Cancer be prevented?
Unfortunately, there is no known way to prevent parathyroid cancer. Also, there is no way to reduce the risk of developing this disease. If anyone in your family has had parathyroid gland conditions, it is a good idea to talk to your doctor.
What questions should I ask my doctor?
If you have parathyroid cancer, you may want to ask your doctor questions like these:
- Has the cancer spread beyond my parathyroid gland?
- What is the best treatment for parathyroid cancer?
- What are the risks and side effects of the treatment?
- Is my family at risk of developing parathyroid cancer?
- Can I get parathyroid cancer again?
- Am I at risk of developing other types of cancer?
- What follow-up care will I need after treatment?
- Should I be aware of symptoms of complications?
Finally, things to remember
Being diagnosed with cancer can be a scary thing. But remember, your medical team is there to help you and show you the best course of treatment. Although surgery is currently the only treatment for parathyroid cancer, research and cancer treatments are constantly improving. There may even be a clinical trial you can take part in. So, don't be afraid to ask your doctor questions about your parathyroid cancer. Make sure everything is explained in a way that you understand.
` Parathyroid Cancer, Parathyroid Glands, Hypercalcemia, Parathyroid Hormone, PTH, Cancer Symptoms, Cancer Treatment, Surgery
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