Treatment of Medullary Carcinoma of the Breast

Together, you and your doctor will develop a treatment plan for medullary carcinoma. Some doctors feel that a true medullary carcinoma may only require surgery without any additional (or adjuvant) treatments, such as chemotherapy or radiation therapy. This is because medullary carcinoma is usually a small tumor that is not aggressive and rarely spreads to the lymph nodes.

Other doctors believe that all cases of medullary carcinoma should be treated like invasive ductal carcinoma. True medullary carcinoma is quite rare and very difficult to diagnose with certainty, and some doctors may want to make sure they are not under-treating the cancer.

Atypical medullary carcinoma would receive the same treatments as invasive ductal carcinoma.

You can work with your doctor to come up with the treatment plan that is right for you, based on the features of the cancer such as tumor size, grade, and whether or not the cancer has spread to any lymph nodes. Your plan may include:

  • Surgery to remove the cancer and, if necessary, any affected lymph nodes. If medullary carcinoma spreads to the lymph nodes, it usually involves no more than 3 nodes. Possible procedures are:
    • Lumpectomy: The surgeon removes only the part of your breast containing the tumor (the “lump”) and some of the normal tissue that surrounds it. Your surgeon may also remove some lymph nodes.
    • Total or simple mastectomy: Removal of the breast without removal of the axillary (underarm) lymph nodes. A sentinel node biopsy may be performed to check the node or nodes closest to the tumor for any signs of cancer spread.
    • Modified radical mastectomy: Surgery to remove the breast, the lining of the chest wall muscle, and some of the lymph nodes under the arm.
  • Chemotherapy: Chemotherapy involves taking anti-cancer medicines in the form of a pill or directly into a vein. The medicines travel through the bloodstream to all parts of the body. The main goal is to destroy any cancer cells that may have broken away from the original tumor.
  • Radiation therapy: Radiation therapy, also called radiotherapy, directs high-energy waves at the area of the body affected by the cancer, such as the chest or the underarm lymph nodes. This directed radiation can destroy any cancer cells that may be left behind.

Most medullary carcinomas test negative for estrogen and progesterone receptors. Therefore, they usually cannot be treated with hormonal therapies such as tamoxifen or aromatase inhibitors, which interfere with estrogen’s ability to promote cancer cell growth. Medullary carcinomas also usually test negative for HER2/neu receptors, and so cannot be treated with Herceptin (chemical name: trastuzumab). Still, be sure to confirm with your doctor whether or not you are a candidate for these therapies.

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Symptoms and Diagnosis of Medullary Carcinoma of the Breast

In this section you can learn about how medullary carcinoma of the breast is detected and diagnosed.

Signs and symptoms

Like other types of breast cancer, medullary carcinoma may not cause any symptoms at first. Over time, a lump can form, and it may be soft and fleshy or somewhat firm to the touch. Most medullary carcinomas are small — less than 2 cm in size. Medullary carcinoma also may cause pain, swelling, redness, or tenderness in the breast.

Diagnosis

Tests that obtain images of the tissue inside the breast, such as mammography and ultrasound, sometimes can find a medullary carcinoma. On these tests, medullary carcinoma appears as a small, well-defined lump. However, some research suggests that mammography is not reliable at detecting medullary carcinomas. One study showed that these cancers were more likely to be found during self-examination or examination of the breasts by a doctor.

Diagnosing medullary carcinoma usually involves a combination of steps:

  • A physical examination of the breasts. Your doctor may be able to feel the lump in the breast.
  • A mammogram to locate the tumor and check for evidence of cancer in other areas of the breast.
  • Ultrasound to obtain additional images of the breast and check for other areas of cancer.
  • Biopsy to remove some or all of the lump to look at it under the microscope. A few cells from the lump can be extracted using a special needle, or the lump can be removed through a small incision. Biopsy is the key to accurate diagnosis, because imaging tests alone can’t tell the difference between medullary carcinoma and other types of breast cancer.

When looked at under a microscope, medullary carcinoma has a number of important features that a pathologist looks for:

  • A clear, well-defined boundary between tumor tissue and normal breast tissue. Medullary carcinoma pushes against the surrounding healthy tissue, but doesn’t grow into it in the same way invasive ductal carcinoma usually does.
  • Large-sized cancer cells with a high-grade appearance, meaning that they look very different from normal, healthy breast cells. However, medullary carcinoma cells do not behave like high-grade cancer cells, which are aggressive and grow and spread quickly. Medullary cancer cells also tend to blend together in a distinctive “sheetlike” pattern; it’s hard to see each cell’s individual membrane (outer lining).
  • Immune system cells (white blood cells called lymphocytes and plasma cells) at the edges of the tumor. Immune system cells work to fight off diseases and any substances they see as a threat to the body. It’s believed that these cells help keep the medullary carcinoma in check, preventing it from growing and spreading quickly.

If the tumor has all of these features, it is considered to be a “true” medullary carcinoma. Sometimes the tumor has only some of these features but not others, or there may be some invasive ductal carcinoma cells mixed in. In these cases, your doctor may call the tumor “atypical medullary carcinoma.”

In addition, medullary carcinoma cells often express a protein called p53. The pathologist may test for p53 to help decide if the cancer is truly medullary.

There are some other key features of medullary carcinoma:

  • Hormone-receptor-negative: Medullary carcinoma usually tests negative for hormone receptors.
  • HER2-negative: Medullary carcinoma also usually tests negative for receptors for the protein HER2/neu.

Diagnosing medullary carcinoma can be challenging. It may be hard to tell the difference between medullary carcinoma cells and cells that make up a usual invasive ductal carcinoma. If you receive a diagnosis of medullary carcinoma, ask your doctor whether the pathologist who looked at the sample has experience diagnosing this type of cancer. You also may want to get a second opinion from another hospital pathology lab.

Medullary Carcinoma of the Breast

Medullary carcinoma of the breast is a rare subtype of invasive ductal carcinoma (cancer that begins in the milk duct and spreads beyond it), accounting for about 3-5% of all cases of breast cancer. It is called “medullary” carcinoma because the tumor is a soft, fleshy mass that resembles a part of the brain called the medulla.

Medullary carcinoma can occur at any age, but it usually affects women in their late 40s and early 50s. Medullary carcinoma is more common in women who have a BRCA1 mutation. Studies have shown that medullary carcinoma is also more common in Japan than in the United States.

Medullary carcinoma cells are usually high-grade in their appearance and low-grade in their behavior. In other words, they look like aggressive, highly abnormal cancer cells, but they don’t act like them. Medullary carcinoma doesn’t grow quickly and usually doesn’t spread outside the breast to the lymph nodes. For this reason, it’s typically easier to treat than other types of breast cancer

On the following pages, you can learn more about:

Follow-up Care for Tubular Carcinoma of the Breast

After treatment, you and your doctor will work together to come up with a schedule of follow-up visits and exams that is right for your situation. Your schedule may include the following tests and exams:

  • You’ll likely have a physical exam and medical history every 4-6 months for 5 years, and then every year after that. If you are taking tamoxifen or other forms of hormonal therapy, you can consult with your doctor about treatment for any side effects you may experience.
  • If you had lumpectomy or breast-conserving surgery, you’ll arrange for a mammogram of the affected breast 6 months after radiation is completed, and then mammography on both breasts every year.
  • If you had mastectomy, you’ll schedule a mammogram of the remaining breast every year. If you are considered high-risk for developing another breast cancer, whether due to strong family history or a positive genetic test for BRCA1 or BRCA2 mutations, your doctor may recommend breast MRI in addition to yearly mammograms.
  • If you’re taking tamoxifen, you’ll have a physical exam and medical history taken by a gynecologist every year, because this medication can increase the risk of cancer of the uterus. Any unusual symptoms, such as abnormal bleeding, should be reported immediately to your doctor. (If you have had a hysterectomy and no longer have a uterus, this recommendation does not apply to you.)
  • If your treatments have put you into menopause early or you have already gone through menopause naturally and are taking an aromatase inhibitor, you’ll need regular monitoring of your bone health with a bone density test. Having lower levels of estrogen in the body, which is a result of early menopause or taking an aromatase inhibitor, can impact bone health.

You may need to have additional tests or more frequent office visits, depending on your individual needs. Ask your doctor what he or she recommends.

Treatment of Tubular Carcinoma of the Breast

You and your doctor will work together to develop your treatment plan. If you have a true tubular carcinoma — made up mostly of the characteristic tube-shaped cells, without other types of breast cancer cells mixed in — it probably won’t require as much treatment as invasive ductal carcinoma does. If invasive ductal carcinoma (IDC) is found in the same or the other breast, the treatment plan will reflect this fact and will likely be different than the treatment plan for tubular carcinoma alone. Tubular carcinoma alone tends to be small and usually does not spread to the lymph nodes.

Your plan can include:

  • Surgery to remove the cancer and any affected lymph nodes. Possible procedures are:
    • Lumpectomy: The surgeon removes only the part of your breast containing the tumor (the “lump”) and some of the normal tissue that surrounds it. Some lymph nodes may be removed as well.
    • Total or simple mastectomy: The surgeon removes the breast without removal of any axillary (underarm) lymph nodes. A sentinel node biopsy may be performed to check the node or nodes closest to the tumor for any signs of cancer spread.
    • Modified radical mastectomy: The surgeon removes the breast, the lining of the chest wall muscle, and some of the lymph nodes under the arm. Since tubular carcinoma is usually small and doesn’t tend to spread, this type of mastectomy is not commonly done.
  • Adjuvant (additional) therapy, such as chemotherapy, radiation therapy, and/or hormonal therapy.
    • Chemotherapy involves taking anti-cancer medicines in the form of a pill or directly into a vein. The medicines travel through the bloodstream to all parts of the body. The main goal is to destroy any cancer cells that may have broken away from the original tumor.
    • Lumpectomy may be followed by radiation therapy, which directs high-energy rays at the area where the cancer was to destroy any remaining cancer cells. Although radiation therapy is considered standard after lumpectomy, some doctors may feel it is not needed for tubular carcinoma, especially in cases where the tumor is very small.
    • Hormonal therapy involves taking medications such as tamoxifen or an aromatase inhibitor, which either block the effects of estrogen or lower the amount of estrogen in the body. Almost all tubular carcinomas are estrogen-receptor-positive, which means that hormonal therapy is likely to be effective.

    Many doctors base their recommendations about hormonal therapy and chemotherapy for tubular carcinoma on the size of the tumor and whether or not there is evidence of cancer in the lymph nodes. Some general guidelines follow — but keep in mind that individual doctors may have different opinions about whether additional treatment is needed for tubular carcinoma.

    • If the tumor is smaller than 1 cm, with no cancer or just a very small amount of cancer in one lymph node: Hormonal therapy may be recommended, but no other treatment is needed.
    • If the tumor is between 1 and 2.9 cm in size, with no cancer or just a very small amount of cancer found in one lymph node: Chemotherapy may be considered along with hormonal therapy.
    • If the tumor is 3 cm or larger and there is spread to the lymph nodes: Chemotherapy is more strongly recommended, with the addition of hormonal therapy.

You and your doctor can discuss all of the risks and benefits of having more treatment beyond surgery. There is some ongoing debate over how necessary additional treatment is in cases of tubular carcinoma. Because this type of tumor is small and slow-growing, some experts feel that more treatment may not have much benefit. This decision likely will come down to what you and your doctor feel is best for you.

Most tubular carcinomas test negative for receptors for the protein HER2/neu, so they usually would not be treated with Herceptin (chemical name: trastumuzab). Still, be sure to confirm with your doctor whether or not you are a candidate for this medication.

Symptoms and Diagnosis of Tubular Carcinoma of the Breast

Tubular carcinoma doesn’t always have obvious symptoms in the breast. Below are symptoms you may have and ways doctors make a diagnosis.

Signs and symptoms

Like other types of breast cancer, tubular carcinoma of the breast may not cause any symptoms at first. Over time, a lump may grow large enough to be felt during breast self-exam or examination by a doctor. Tubular carcinomas are usually small — 1 cm or less in diameter — and feel firm or hard to the touch.

Diagnosis

Most tubular carcinomas are first detected either during breast examination or by a screening mammogram. On a mammogram, a tubular carcinoma looks like a small mass with an irregular shape. Further testing is needed to tell for sure whether it is a tubular carcinoma.

Diagnosing tubular carcinoma usually involves a combination of steps:

  • A physical examination of the breasts. Your doctor may be able to feel the lump in the breast, or you may feel it yourself during a breast self-exam.
  • A mammogram can locate the tumor and check for evidence of cancer in both breasts.
  • MRI, ultrasound, or both may be used to obtain additional images of the breasts and check for other areas of cancer.
  • Biopsy. A biopsy involves taking out some or all of the tumor to look at it under the microscope. The sample can be taken by inserting a special needle or making a small incision. Biopsy is the key to accurate diagnosis, because imaging tests alone can’t distinguish tubular carcinoma from other types of breast cancer and benign (noncancerous) breast conditions.

When a pathologist examines the tumor samples under a microscope, he or she looks for the tube-shaped structures that give tubular carcinoma its name. Experts generally agree that the vast majority of the tumor must have this characteristic appearance in order to be called tubular carcinoma. The more “tubular” it is, the less likely it is to spread and the easier it should be to treat.

Tubular carcinoma is sometimes found near other more common types of breast cancer. Ductal carcinoma in situ (or DCIS, a type of cancer that begins and remains within the milk duct) is often found next to or mixed in with the tubular carcinoma. Less often, doctors may find a lobular carcinoma in situ (LCIS, a precancerous condition confined to the lobule) or even an invasive lobular carcinoma.

Studies have shown that about 10-15% of women found to have tubular carcinoma in one breast also have cancer in the other breast. The cancer in the other breast is usually invasive ductal carcinoma (IDC), the most common type of breast cancer. This is why it is so important to have both breasts checked thoroughly.

As with the other rare subtypes of breast cancer, diagnosing tubular carcinoma takes special skill. You may want to ask for a second opinion if this is your diagnosis.

There are some other important features of a true tubular carcinoma of the breast:

  • Hormone-receptor-positive: Studies show that tubular carcinoma tests positive for estrogen receptors 80-90% of the time. Tubular carcinoma also tests positive for progesterone receptors in most cases — about 68-75% of the time, according to one study.
  • HER2-negative: Tubular carcinoma usually tests negative for receptors for the protein HER2/neu.

Tubular Carcinoma of the Breast

Tubular carcinoma of the breast is a rare subtype of invasive ductal carcinoma (cancer that begins inside the milk duct and spreads beyond it). Tubular carcinoma accounts for about 1-2% of all breast cancer cases. In this type of cancer, the tumor is usually small and made up of tube-shaped cells that are low grade. “Low grade” means they look somewhat similar to normal, healthy cells and tend to grow slowly.

Tubular carcinoma of the breast is less likely to spread outside the breast than other types of breast cancer. It’s also easier to treat.

Studies have found that the average age of diagnosis for tubular carcinoma ranges from the mid-40s to late 60s.

On the following pages, you can learn more about:

Symptoms and Diagnosis of Tubular Carcinoma of the Breast

Treatment of Tubular Carcinoma of the Breast

Follow-up Care for Tubular Carcinoma of the Breast