Thyroid cancer
From MayoClinic.com Special to CNN.com

Introduction

 

The thyroid is a butterfly-shaped gland located at the base of your neck, just below your Adam's apple. Although the thyroid gland is small, it produces hormones that regulate every aspect of your metabolism, from your heart rate to how quickly you burn calories.

Sometimes you may develop one or more solid or fluid-filled lumps called nodules in your thyroid. Most of these are noncancerous (benign) and cause no symptoms. But a small percentage are cancerous (malignant), and serious complications are possible.

The prognosis is often excellent for a cancerous thyroid nodule. The most common types of thyroid cancer can often be completely removed with surgery. But the important first step is to know the symptoms and see your doctor.

 

Signs and symptoms

 

Most often, you won't have signs and symptoms in the early stages of thyroid cancer, but as the cancer grows, you may experience one or more of the following:

Having one or more of these symptoms doesn't necessarily mean you have thyroid cancer. Other conditions — including a benign thyroid nodule, an infection or inflammation of the thyroid gland, and a benign enlargement of the thyroid (goiter) — can cause similar problems, all of which are highly treatable.

 

Causes

 

Your thyroid gland is composed of two lobes that resemble the wings of a butterfly separated by a thin section of tissue called the isthmus. The thyroid takes up iodine from the food you eat and uses it to manufacture two main hormones, thyroxine (T-4) and triiodothyronine (T-3). These hormones maintain the rate at which your body uses fats and carbohydrates, help control your body temperature, influence your heart rate and regulate the production of protein. Your thyroid gland also produces calcitonin, a hormone that regulates the amount of calcium in your blood.

 

 

The thyroid contains two main types of cells. Follicle cells make the thyroid protein thyroglobulin and produce and store thyroxine and triiodothyronine. Other cells, called C cells (parafollicular cells), produce calcitonin. The distinction is important because each type can give rise to different types of cancer.

Papillary and follicular cancers develop in follicle cells. They account for the great majority of thyroid cancers, can usually be completely removed with surgery and generally result in an excellent prognosis. Medullary cancer, on the other hand, arises in the thyroid's C cells and is generally more aggressive and harder to treat than papillary and follicular cancers are.

The types of thyroid cancer include:

Papillary cancer (papillary carcinoma, papillary adenocarcinoma)
This is the most common type of thyroid cancer. It develops from thyroid follicle cells and usually appears as a single mass in one lobe of the thyroid. Anyone, including children, can develop papillary cancer, but it's most common in women who are between 30 and 50 years of age.

Although most papillary cancers grow slowly, they often spread to the lymph nodes early in the course of the disease. This usually doesn't affect the outlook for recovery, which is generally excellent when the cancer is small and its spread limited to the lymph nodes in your neck. The prognosis isn't as positive for people with very large tumors or in the rare cases when papillary cancer has invaded tissues other than the lymph nodes. But even papillary tumors that have spread to the lungs or bone often can be successfully treated with radioactive iodine (radioiodine).

Follicular cancer (follicular carcinoma, follicular adenocarcinoma)
This type of cancer is more aggressive and affects a slightly older population than does papillary cancer. Follicular tumors don't usually spread to the lymph nodes but are likely to invade the veins and arteries within the thyroid. From there, they may spread to organs such as your lungs and bone.

Medullary cancer (medullary carcinoma)
Rather than arising from follicle cells, this type of thyroid cancer develops in calcitonin-producing C cells. These tumors usually make calcitonin along with carcinoembryonic antigen (CEA) — a protein produced by certain cancers. Both are released into the bloodstream and can be detected by blood tests. But in many cases, medullary cancer may spread to the lymph nodes or other organs before a lump is detected or blood tests are performed.

There are three main types of medullary cancer:

Anaplastic cancer (anaplastic carcinoma)
This rare form of thyroid cancer is sometimes called undifferentiated cancer because it looks very different from normal thyroid tissue under a microscope. It appears to develop from an existing, undiagnosed papillary or follicular cancer. Anaplastic cancer is extremely aggressive, spreads rapidly to the lymph nodes and trachea, and then to other organs, especially the lungs and bone. For that reason, it's often not curable surgically by the time it's diagnosed. Unfortunately, other therapies, such as radiation, aren't usually successful in controlling anaplastic cancer.

Thyroid lymphoma
This rare type of cancer doesn't develop from thyroid follicular cells or C cells. Instead, it starts in immune system cells called lymphocytes. Although most lymphomas begin in the lymph nodes, some occasionally appear in other organs, such as the thyroid.

What causes thyroid cancer?
Healthy cells grow and divide in an orderly way. This process is controlled by DNA — the genetic material that contains the instructions for every chemical process in your body. When DNA is damaged or altered, changes occur in these instructions. One result is that cells may begin to grow out of control and eventually form a tumor — a mass of malignant cells.

In the case of thyroid cancer, DNA damage can occur from exposure to environmental contaminants such as radiation, from the aging process or, in medullary cancers, from genetic causes:

 

Risk factors

 

Although the exact cause of many cases of thyroid cancer isn't known, certain factors increase your risk:

 

When to seek medical advice

 

See your doctor if you develop any of the symptoms of thyroid cancer, including a lump in your neck near your Adam's apple, hoarseness, or trouble swallowing or breathing. And don't hesitate to talk to your doctor if you think you may be at risk of thyroid problems or are worried about radiation treatments you received in childhood.

 

Screening and diagnosis

 

Although you may see or feel a lump (nodule) in your thyroid yourself — usually just to the lower right or left of your Adam's apple — it's more likely that your doctor will discover a lump during a routine medical exam. You're usually asked to swallow while your doctor examines your thyroid because the thyroid moves up and down during swallowing, making nodules easier to feel.

Sometimes a thyroid nodule is detected as an incidental finding when you have an imaging test to evaluate another condition in your head or neck. Nodules detected this way are usually too small to be found during a physical exam.

To help determine whether a nodule is malignant, you may have one or more of the following tests:

Staging tests
If you receive a diagnosis of thyroid cancer, you're likely to have tests to help determine whether the cancer has spread (metastasized) — a process known as staging. The stage of cancer helps your doctor determine the best course of treatment and the outlook for your recovery. The staging tests you have may vary, depending on the type of thyroid cancer.

 

 

Screening tests
If you have medullary cancer, consider having DNA testing, which checks a blood sample for the known genetic defects that cause familial and MEN-associated medullary thyroid cancer. It's best to meet with a genetic counselor to discuss what a positive or negative test result may mean for your family.

If you have medullary thyroid cancer but don't test positive for the RET gene, it's still important that your close family members have their calcitonin levels tested. This is generally done using a calcium infusion test. Although the calcitonin level of healthy people rises slightly after an injection of calcium, it's much higher in people with medullary thyroid cancer.

The calcium infusion test usually takes between 15 and 20 minutes and is done on an outpatient basis. You'll have a small amount of blood drawn before the injection of calcium and again at two, five, 10 and 15 minutes after the injection.

 

Complications

 

Thyroid tumors can lead to a number of complications, including:

 

Treatment

 

Surgery is the main treatment for most types of thyroid cancer, but other therapies may vary, depending on the type of thyroid cancer you have.

Papillary and follicular thyroid cancers
The best type of surgery for follicular and papillary thyroid cancers was once a matter of debate. Now most experts agree that the optimal treatment is near-total thyroidectomy — an operation that removes practically the entire thyroid with the exception of small rims of tissue around the parathyroid glands to reduce the risk of parathyroid damage. If you have enlarged lymph nodes as a result of thyroid cancer, your operation may be extended to remove the affected lymph nodes. In some cases, this may mean exploring and removing enlarged lymph nodes on both sides of your neck. Cancer is less likely to return or spread after thyroidectomy than after less complete operations, and in experienced hands, the risks of the surgery are low.

Surgical treatment of follicular cancer is more complicated than that of papillary cancer because follicular cancer usually can't be diagnosed until the affected tissue is examined. Sometimes this occurs during surgery using a technique called frozen section, which takes less than 10 minutes to complete and which is performed while you're still anesthetized. When this procedure isn't available, surgeons are likely to remove the lobe of the thyroid that contains the nodule (lobectomy) and send it to a pathologist, who examines it under a microscope. If the nodule is malignant, the next step is near-total thyroidectomy.

After any type of surgery for thyroid cancer, you'll need to take the thyroid hormone medication levothyroxine (Levothroid, Synthroid) for life. This has two benefits: It supplies the missing hormone your thyroid would normally produce, and it suppresses the pituitary's production of TSH, which signals your thyroid to manufacture hormones. High TSH levels could conceivably stimulate any remaining cancer cells to grow.

You'll likely have blood tests to check your thyroid hormone levels every few months until your doctor finds the proper dosage for you. Too much hormone can cause unintended weight loss, palpitations, tremors, osteoporosis and frequent bowel movements. Too little may lead to weight gain, sensitivity to cold, and dry skin and hair.

Other treatments for papillary and follicular cancers include:

Follow-up care after treatment for follicular or papillary thyroid cancer is important. Thyroid cancer can recur as many as 20 or 30 years after the original diagnosis, although if you've remained cancer-free for five years, the recurrence rate is low.

Still, you'll have periodic blood tests to monitor your level of thyroglobulin, a protein that stores thyroid hormone. Elevated levels of this hormone could indicate that the cancer has returned. You may also have imaging tests or other tests that help your doctor check for a recurrence of cancer.

Medullary thyroid cancer
This type of cancer usually occurs in both lobes of the thyroid gland and often involves multiple tumors. For that reason, the best treatment is total thyroidectomy. And because medullary cancer has often spread to the lymph nodes by the time it's diagnosed, you may have lymph nodes removed (dissection) on both sides of your neck. When the cancer hasn't metastasized, the outlook after surgery is excellent. If the cancer has spread to other organs, treatment depends on several factors, including the size of the tumor, how quickly it's growing, and the extent of the spread. For example, your doctor might choose not to surgically remove a small tumor in the liver, lung or bone. Such tumors sometimes grow slowly for years without causing any symptoms. Large or rapidly growing tumors, on the other hand, may need surgery or other treatment. In that case, you and your doctor will work together to decide on the best type of therapy. Radioiodine treatments aren't an option for people with medullary cancer because thyroid C cells don't absorb iodine, but you may receive external radiation or chemotherapy, which uses drugs to kill cancer cells. Not every person with medullary thyroid cancer responds to chemotherapy, but in some cases a combination of cancer drugs may shrink tumors or slow their growth. The encouraging news is that although medullary thyroid cancers can be aggressive, some grow slowly — sometimes for years — without causing major symptoms. Follow-up care includes regular physical examinations and blood tests to check your calcitonin and CEA levels.

Anaplastic thyroid cancer
The most aggressive and fastest growing type of thyroid cancer, anaplastic cancer often can't be helped by surgery by the time it's diagnosed. Radiation or chemotherapy may shrink tumors slightly and make you more comfortable. But because no treatment can eliminate advanced anaplastic cancer, you may want to consider participating in a clinical trial. This is a study that tests new forms of therapy — typically new drugs or surgical procedures, or novel treatments such as gene therapy. If the therapy proves to be safer or more effective than current treatments, it becomes the new standard of care. But treatments used in clinical trials haven't been shown to be effective. They may have serious or unexpected side effects, and there's no guarantee you'll benefit from them. On the other hand, cancer clinical trials are closely monitored to ensure that they're conducted as safely as possible. And they offer access to treatments that wouldn't otherwise be available to you.

If you're interested in finding out more about clinical trials, talk to your doctor. You can also call the National Cancer Institute's Cancer Information Service at (800) 4-CANCER, or (800) 422-6237. The call is free, and trained specialists are available to answer your questions.

 

Prevention

 

It's often not possible to prevent thyroid cancer. But the following measures may reduce or eliminate your risk:

 

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