Medullary Carcinoma

Related Terms: Cancer, Lymphedema

We are all familiar with cancers such as breast cancer, lymphomas, prostate, melanoma etc from all of which lymphedema can develop from its diagnostic workup or treatments, but there are lesser known malignancies that also give rise to lymphatic damage. One of these is Medullary Carcinoma.

This is a cancer that is is composed mainly of epithelial (Membranous tissue composed of one or more layers of cells separated by very little intercellular substance and forming the covering of most internal and external surfaces of the body and its organs.) elements and most commonly starts in either the breast or thyroid gland.

It is named from its appearance, which resembles the tissues of the “Medulla oblongata” region of the brain stem.

In terms of breast cancer, it represent about 5% of all BC cancers and s more common in women who carry the BRCA-1 mutation.

Medullary Carcinoma of the Breast

Signs and Symptoms

You may feel a lump that feels like a thick, spongy area of breast tissue. The tumor may also feel smooth-sided, like a breast cyst.

Diagnosis

A diagnostic workup for medullary carcinoma may include:

(1) A physical examination of the breasts, checking for any possible lumps

(2) A mammogram to locate the actual tumor

(3) An ultrasound in checking for other areas of cancer

(4) Biopsy - removal of tissue or the entire lump to be studied by a pathologist. The biopsy can include either an invasive surgical procedure, a small incision and/or small needle biopsy. The doctor will need to determine the best procedure. A Stereotactic (Mammographically Guided) Breast Biopsy may also be used. In this procedure, a special machine uses ionizing radiation to help guide the readiologists instruments to the site of the tumor.

A FDG PET scan may also be useful in searching for possible metastases of the cancer.

Treatment

Your treatment plan will be determined by the extent of the cancer. Treatments can include surgery only, chemotherapy or radiation.

Surgeries may involve a lumpectomy, a total or simply mastectomy, or a modified radical mastectomy.

Medullary Carcinoma of the Thyroid

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

There are two forms of MTC:

Sporadic MTC, which does not run in families. Most MTCs are sporadic. This form mainly affects older adults.

Inherited MTC runs in families.(1)

Symptoms:

Difficulty in breathing, Cough, Cough with blood, Diarrhea, Enlarged Thyroid (Goiter), Lump on thyroid gland.

Patients with locally advanced disease may present with hoarseness, dysphagia, and respiratory difficulty. Patients may also present with various paraneoplastic syndromes, including Cushing or carcinoid syndrome.

If there has been distant metastases, there may be symptoms of weight loss, lethargy, and bone pain. (2)

Causes:

Medullary carcinoma of the thyroid has a genetic association with multiple endocrine neoplasia (MEN)2A and 2B. If you have a family history of familial medullary thyroid cancer, multiple endocrine neoplasis and familial adenomatouspolyposis you have an increased risk of developing medullary carcinoma fo the thyroid (MTC).

Diagnostic Tests:

Diagnostic tests include a physical exam to check for thyroid lumps amd lymph node swelling; blood tests, including measuring the level of thyroid-stimulating hormone (TSH); imaging tests such as an ultrasound, or PET and finally an ultrasoun-guided needle biopsy to remove cells from the thyroid for study by a pathaologist.

Treatments:

Your physician/oncologist will develop a specific treatment plan based on your own personal situation. This treatment may include surgery to remove all or part of the thyroid (thyroidectomy), lymph node removal of the neck may be necessary for testing as well. The complications that can arise from the surgery include possible damage to surrounding nerves, bleeding, infection and of course lymphedema if the lymph nodes have been removed and/or radiated.

After the surgery, you will be given thyroid hormone medication for life. This medicine has two benefits and it suppresses the production of thyroid-stimulating hormone (TSH) from your pituitary gland. High TSH levels could stimulate any remaining cancer cell to grow.

Radioactive iodine treatment uses a form of iodine that is radioactive. It comes in a capsule or liquid that you can swallow. Side effect may include nausea, dry mouth, dry eyes, altered sense of taste or smell.

External radiation therapy may also be given. This treatment is typically administered for a few minutes at a time five days a week for about six weeks. This is used to treat thyroid cancer that has spread to the bones.

Finally, chemotherapy may also be used. This is given as an infusion to kill the cancer cells

Lymphedema

Arm lymphedema (arm swelling) develops in between 25% - 35% of breast cancer patients as a result of lymph node removal, radiation, or chemotherapy.

It can also develop with thyroid cancer, however, lymphedema of the neck is a distinct possiblility with damage to, or removal of lymph nodes in the neck.

For further information on lymphedema, please see our pages:

Arm Lymphedema, Lymphedema of the Head and/or Lymphedema of the Neck.

(1) Medline Plus

(2) eMedicine