Definition of Medullary carcinoma of thyroid:

Medullary carcinoma of the thyroid is cancer of the thyroid gland that starts in cells that release a hormone called calcitonin. Such cells are called "C" cells.

Causes, incidence, and risk factors:

The cause is unknown. Unlike other types of thyroid cancer, medullary carcinoma of the thyroid (MTC) is believed to be related to radiation therapy (a type of cancer treatment).

There are two forms of MTC:

• Sporadic MTC does not run in families. Most MTCs are sporadic. This form mainly affects older adults.
• Inherited MTC runs in families.

You have an increased risk of medullary carcinoma of the thyroid if you have:

• A family history of multiple endocrine neoplasia (MEN)
• A prior history of pheochromocytoma, mucosal neuromas, or hyperparathyroidism

Other types of thyroid cancer include:

• Anaplastic carcinoma of the thyroid
• Follicular tumor of the thyroid
• Papillary carcinoma of the thyroid
• Thyroid lymphoma

Symptoms:

• Breathing problems due to narrowing of airways
• Cough
• Cough with blood
• Diarrhea
• Goiter
• Lump on thyroid gland

Signs and tests:

The health care provider will perform a physical exam. Lymph nodes in the neck may be swollen. Thyroid function tests are usually normal. However, an examination of the thyroid may reveal single or multiple nodules (lumps).

Other tests that may be used to diagnose medullary carcinoma of the thyroid include:

• Calcitonin blood test
• CEA blood test
• Thyroid biopsy
• Thyroid scan
• Ultrasound of the thyroid

Treatment:

Treatment involves surgery to remove the thyroid gland and surrounding lymph nodes. Because this is an uncommon tumor, surgery should be performed by a surgeon who is familiar with this type of cancer.

Chemotherapy and radiation do not work very well for this type of cancer. Radiation is used in some patients after surgery. There are a number of new treatments currently being investigated in clinical trials.

Support Groups:

For additional information, see cancer support groups.

Expectations (prognosis):

Approximately 86% of those with medullary carcinoma of the thyroid live at least 5 years after diagnosis. The 10-year survival rate is 65%.

Complications:

Complications may include:

• Cancer spreads to other areas of the body
• Parathyroid glands are accidentally removed during surgery

Calling your health care provider:

Call your health care provider if you have symptoms of medullary carcinoma of the thyroid.

Prevention:

Prevention may not be possible. However, being aware of your risk factors, especially your family history, may allow for early diagnosis and treatment.