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Medullary Thyroid Cancer

What is medullary thyroid cancer?

Medullary thyroid cancer (MTC), also known as medullary thyroid carcinoma, is a type of thyroid cancer.

The thyroid gland is located just below the throat in the front of the neck. The thyroid makes hormones that control body functions. MTC starts in parafollicular C cells of the thyroid gland. These cells make a hormone called calcitonin.

Medullary thyroid cancer is rare in children. Most often, childhood MTC is linked to an inherited change in the RET gene. This causes a condition called familial MTC or multiple endocrine neoplasia type 2 (MEN2 syndromes).

Surgery to remove the thyroid gland is the main treatment for medullary thyroid cancer. For children with a genetic predisposition, doctors may remove the thyroid before the cancer develops.

Patients need ongoing care from a team of specialists to manage hormones and watch for the return of cancer.

Graphic of an adult female body with layover of organs visible and the thyroid gland is highlighted and labeled.

The thyroid gland is a butterfly-shaped organ located at the base of the throat in the front of the neck. It is arranged in two lobes, one on the right side and one on the left side.

Symptoms of medullary thyroid cancer

Signs and symptoms of MTC include:

  • A nodule or lump in the thyroid gland
  • Swollen lymph nodes in the neck
  • Breathing problems
  • Problems swallowing or pain when swallowing
  • Hoarseness

Your child may not have any symptoms of MTC. The doctor may find it during your child's regular physical exam.

Risk factors for medullary thyroid cancer

MTC is rare in children, but certain gene changes can increase risk. Parents may sometimes pass down gene changes to their children.

A genetic disorder called hereditary or familial MTC can run in families. In this disorder, there is a change (mutation) in the RET gene so it does not work properly. A child may inherit this mutation from either parent. Or your child may be the first person in your family with this gene change.

RET mutations can occur in a condition known as multiple endocrine neoplasia type 2 (MEN2). MEN2 increases risk of tumors and hormone problems including medullary thyroid cancer, pheochromocytoma, and hyperparathyroidism.

If your child has a RET mutation, your family should get genetic counseling and genetic testing.

Diagnosis of medullary thyroid cancer

Doctors use several types of tests to diagnose medullary thyroid cancer. Tests may include:

  • Physical exam and health history. This includes a family health history to check for an inherited cancer risk.
  • Blood tests to learn about hormones and gene changes
  • Tests for tumor markers
    • Calcitonin is a hormone produced by the thyroid gland. It may be higher in medullary thyroid cancer.
    • Carcinoembryonic antigen (CEA) is a protein that is normally low in the blood. CEA may be higher in medullary thyroid cancer.
  • Imaging tests to find tumors, measure their size, and see if the cancer has spread. Doctors use these details to plan the next steps for diagnosis and treatment. 
    • Ultrasound
    • Computed tomography (CT scan)
    • Magnetic resonance imaging (MRI) 
    • PET-CT scan with dotatate tracer. A dotatate scan takes pictures of the whole body to look for neuroendocrine tumors. Your child gets a small amount of radioactive material (tracer). The tracer goes into the blood and travels through the body. After the tracer is absorbed, the scanner detects radiation to make images. The tracer highlights neuroendocrine cells that take up the tracer and may indicate tumors.  
  • Biopsy to see if there are cancer cells. A fine needle aspiration allows doctors to take a sample of thyroid tissue using a thin needle inserted through the skin.

Stage of medullary thyroid cancer

The stage of cancer depends on the tumor size and if the cancer has spread to lymph nodes or other parts of the body.

Stage Extent of disease
Stage 1 Tumor is 2 centimeters (cm) or less across. There is no spread of disease.
Stage 2 Tumor is larger than 2 cm across. It is only in the thyroid. There is no spread to lymph nodes or other parts of the body.
Stage 3 The tumor may have grown slightly outside the thryoid gland, and the disease has spread to nearby lymph nodes in the neck.
Stage 4 The disease is morderate or advanced. The tumor has spread outside of the thyroid into other tissue. The tumor has spread to nearby lymph nodes or distant body parts.

Treatment of medullary thyroid cancer

It is important that a team of specialists treat children with MTC. The treatment depends on gene mutations and other health conditions. The care team will focus on:

  • Cure
  • Lowering the risk of disease recurrence
  • Reducing treatment side effects

All patients with MTC need ongoing care and long-term follow-up.

 

  1. Surgery

    The main treatment for medullary thyroid cancer is surgery to remove the thyroid gland. This is called a total thyroidectomy. The surgeon may also remove nearby lymph nodes and other tissues, known as neck dissection. These surgeries help stop the cancer from coming back.

    An experienced surgeon should perform the surgery. Look for one who does this type of surgery regularly in pediatric patients. This lowers the risk of problems afterward.

    The thyroid makes hormones that control some body functions. After thyroid removal, the body will not make these hormones. To replace the missing hormone, your child will need to take thyroid hormone replacement medication (levothyroxine) for the rest of their life. Physical therapy can help your child with neck strength and movement after surgery.

    Preventive surgery for MEN syndromes

    The best way to treat MTC is to prevent the spread of the disease. Children with genetic mutations are at higher risk for developing this cancer. Doctors may do surgery to remove the thyroid gland before cancer is found (prophylactic thyroidectomy).

    Doctors recommend this surgery for children younger than 5 years who have a MEN2A mutation. A child with a MEN2B mutation should have a thyroidectomy before age 1. In patients with the MEN2B mutation, MTC develops early and is aggressive.

    Risks of thyroid surgery

    There is a risk of damage to neck structures during surgery, such as the nerves and parathyroid glands.

    Hypoparathyroidism due to damage to parathyroid glands

    Parathyroid glands are small and lie behind the thyroid. They keep calcium at normal levels in the body. Sometimes, parathyroid glands are harmed during surgery. The parathyroid glands may become "stunned" after this surgery, even if they are not damaged.

    Patients may get a condition known as transient post-surgical hypoparathyroidism. Symptoms of this condition include:

    • Low levels of calcium in the blood (hypocalcemia)
    • Numbness and tingling in the hands, feet, and face
    • Muscle cramps

    Doctors treat this condition with vitamin D (calcitriol) and calcium supplements until these problems stop. Hypoparathyroidism may be brief or permanent.

    Nerve or vocal cord problems

    Another risk of thyroid surgery is nerve damage to the recurrent laryngeal nerve. This nerve helps the vocal cords move for speech. Nerve problems after thyroid removal are rare. The more experienced the surgeon, the lower the risk of problems. During the surgery, the surgeon may also use a special breathing tube to prevent injury to the nerves.

    An otolaryngologist will do a "scope" procedure to check the vocal cords before and after surgery. The doctor will gently place a small, flexible camera into your child's nose to see the vocal cords and check their function.

    Vocal cord injuries during surgery are often temporary. But some patients may need a longer recovery time, even a year or longer. Patients may need more treatment if their voice changes or they have problems breathing or swallowing.

    The doctor can inject temporary material into the vocal cords to help them work better. In some cases, the patient may need surgery to improve vocal cord function.

  2. Targeted Therapies

    Doctors are studying targeted therapies to treat MTC. These medicines work on certain features of cancer cells. These treatments may kill cancer cells or stop them from growing. One example is a group of drugs known as kinase inhibitors.

    Unlike some other thyroid cancers, medullary thyroid cancer does not take up iodine. Therefore, radioactive iodine is not used for imaging and treatment of MTC.

    Patients with disease that comes back may consider treatment in a clinical trial.

Prognosis for medullary thyroid cancer

MTC is a rare cancer, and many factors affect long-term outcomes. These factors include:

  • Stage of the disease when it was diagnosed
  • How much surgery your child had
  • Whether the disease has spread to other places, such as the lungs or bones
  • Gene mutations or inherited conditions that increase the risk for other cancers
  • Calcitonin levels after surgery

Support for patients with medullary thyroid cancer

After treatment, patients need lifelong monitoring and follow-up care from a team of specialists. This is important because:

  • There is a risk that the cancer could come back (recurrence)
  • Hormone function is affected
  • Gene changes may increase risk for other health problems or cancers

Monitoring for recurrence and follow-up care

Your child's long-term monitoring may include:

  • Physical exams
  • Neck ultrasound
  • Blood tests to monitor calcitonin and carcinoembryonic antigen (CEA) levels
  • Tests to make sure thyroid stimulating hormone (TSH) stays in the normal range
  • Managing medicines including thyroid hormone replacement (levothyroxine)

The type and frequency of tests and medical visits depend on your child's needs, thyroid cancer type, and the stage. Specialists will monitor your child and watch for signs of other cancers related to any known genetic mutations.

Psychosocial care after thyroid cancer

Care team members from psychology, child life, social work, and other areas can help your child cope with their illness. Genetic counselors can help you understand inherited cancers and available support services.
Concerns that patients and families may have include:

  • Adjusting to daily medication use
  • Worry about genetic conditions and future risks for cancer
  • How genetic conditions might affect future children or other family members
  • Body image concerns due to surgical scars
     

Health after cancer

To stay well, all cancer survivors should take steps to live a healthy lifestyle. Get regular physical checkups and screenings by a health care provider who understands cancer survivorship issues.

Your care team may prepare a survivorship care plan. This plan is a complete record of your child's treatment, medical concerns, and health screenings they need.

Questions to ask your care team

  • What is the type and stage of cancer?
  • Is the cancer related to a genetic condition?
  • Does my child or family need genetic tests and genetic counseling?
  • What are the treatment options for my child’s cancer?
  • What are the possible risks of side effects of these treatments?
  • If my child needs surgery, how often does the surgeon perform these surgeries in children?
  • Will my child need to stay in the hospital for treatment?
  • What long-term monitoring and follow-up does my child need?

Key points about medullary thyroid cancer

  • Medullary thyroid carcinoma (MTC) is a rare type of thyroid cancer.
  • Certain gene changes increase the risk of developing MTC.
  • In children, MTC is most often seen as part of a genetic syndrome.
  • In some cases, the thyroid gland may be removed to prevent MTC before there are any signs of cancer.
  • MTC treatment includes surgery to remove the thyroid gland and lymph nodes that may have cancer.
  • After surgery, MTC patients need lifelong care, monitoring, and thyroid hormone replacement therapy.

Find more information

—
Reviewed: December 2022

Previous
Differentiated Thyroid Cancer

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