Inflammatory Breast Cancer

  • Medical Author:
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

  • Medical Editor: Jerry R. Balentine, DO, FACEP
    Jerry R. Balentine, DO, FACEP

    Jerry R. Balentine, DO, FACEP

    Dr. Balentine received his undergraduate degree from McDaniel College in Westminster, Maryland. He attended medical school at the Philadelphia College of Osteopathic Medicine graduating in1983. He completed his internship at St. Joseph's Hospital in Philadelphia and his Emergency Medicine residency at Lincoln Medical and Mental Health Center in the Bronx, where he served as chief resident.

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Inflammatory breast cancer facts

  • Inflammatory breast cancer is breast cancer that has spread to the lymphatic system, causing symptoms of inflammation (redness, swelling, tenderness) in the breast.
  • Symptoms of inflammatory breast cancer include
  • As with other types of breast cancer, a tissue biopsy is done to confirm the diagnosis of breast cancer.
  • Inflammatory breast cancers are stage III or stage IV at the time of diagnosis due to their spread into the lymphatic system.
  • Treatment of inflammatory breast cancers is multimodal and involves surgery, chemotherapy, and radiation therapy.
  • Inflammatory breast cancer is more aggressive and tends to have a worse prognosis than other types of breast cancer.
  • Targeted therapies such as trastuzumab (Herceptin) or hormonal therapies may also be given, depending upon whether or not the tumor cells express hormone receptors or the HER2 protein.
  • Survival rates for inflammatory breast cancer are not as favorable as those for other types of breast cancer.

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Breast Cancer Diagnosis and Treatment

Breast Cancer Symptoms & Signs

Breast cancer does not always produce symptoms, but when symptoms do occur, a lump or mass in the breast is the most common symptom. Other possible symptoms include nipple discharge or redness, changes in the skin such as puckering or dimpling, and swelling of part of the breast. Certain types of cancers known as inflammatory breast cancers may produce redness and warmth of the affected breast, but these are not common symptoms of breast cancer.

What is inflammatory breast cancer?

Inflammatory breast cancer is a rare and aggressive form of breast cancer. It is typically a very aggressive disease and is called "inflammatory" because the cancer cells block the lymphatic vessels, resulting in changes in the breast (swelling and redness) that make the breast appear to be inflamed. Over 230,000 women in the United States are diagnosed with breast cancer each year; inflammatory breast cancers make up only 1%-5% of breast cancers.

What are the causes and risk factors for inflammatory breast cancer?

The causes and risk factors for inflammatory breast cancer are the same as those for breast cancer in general. Certain genetic mutations and family history of breast cancer are two of the known risk factors. However, inflammatory breast cancer tends to affect certain women more frequently than others (See "How is inflammatory breast cancer different from other breast cancers?").

How is inflammatory breast cancer different from other breast cancers?

Inflammatory breast cancer is typically an aggressive form of cancer that spreads rapidly. Because it involves the lymphatic system and has invaded the lymph vessels at the time of diagnosis, it is already at a more advanced stage (or extent of spread) than many breast cancers when it is discovered. This type of breast cancer is usually found in women at a younger age than most breast cancers; the median age for diagnosis of inflammatory breast cancer is 57 years compared to 62 years for all breast cancers.

Inflammatory breast cancer is more common in African American women than in Caucasian women and is diagnosed at an earlier age. In Africa American women, the median age at diagnosis of inflammatory breast cancer is 54 years, compared with 58 years for Caucasian women. Inflammatory breast cancer is also more common in obese women than in women with normal body weight.

Inflammatory breast cancers often are hormone receptor negative, meaning that their cells do not have receptors for estrogen or progesterone on the surface. This means that therapies (such as tamoxifen [Nolvadex]) that target estrogen-driven tumor growth are unlikely to be effective.

What are the signs and symptoms of inflammatory breast cancer?

Unlike most breast cancers, which may or may not cause any symptoms, inflammatory breast cancer produces signs and symptoms of inflammation in the breast tissue, including

  • swelling and skin redness that affect a large part (at least one-third) of the breast,
  • pitting,
  • dimpling,
  • bruising,
  • orange peel (peau d'orange) appearance due to the buildup of lymphatic fluid, and
  • nipple may be pulled inward (inverted).

Other possible symptoms and signs include

  • a rapid increase in size of the breast,
  • heaviness,
  • burning, and
  • tenderness.

Sometimes, a mass or lump can be felt in the breast, but commonly no mass can be felt. Enlarged lymph nodes may be present in the underarm or near the collarbone.

While the symptoms of inflammation in the breast are characteristic of inflammatory breast cancer, they can also arise due to infections and other conditions, so any unusual symptoms or changes in the breasts should be evaluated by a medical professional.

What types of doctors treat inflammatory breast cancer?

As with all types of breast cancers, surgeons (including surgical oncologists and breast surgeons) are involved in the treatment plan for removal of the tumor and sometimes lymph nodes. Further treatment is done by oncologists (specialists in treating cancers), including both medical oncologists and radiation oncologists. These providers work as a team and will call in additional specialists as needed (radiation oncologist).

What tests and exams do health-care professionals use to diagnose inflammatory breast cancer?

Mammography and imaging tests

Screening mammography has made it possible to detect many breast cancers before they produce any signs or symptoms. However, inflammatory breast cancer produces symptoms, so in the case of this cancer, mammography may be used to evaluate the breast when symptoms are present or to determine the location for a breast biopsy. Due to the rapid spread of the cancer, this cancer is sometimes found between the routine mammogram exams. Other imaging tests, including MRI, ultrasound, PET scans, and CT scans may be used to evaluate the breast if inflammatory breast cancer is suspected. A bone scan to look for spread (metastasis) to the bones may also be performed if a diagnosis of inflammatory breast cancer is confirmed.

Definitive diagnosis

Even if imaging tests show an abnormality or are suspicious for breast cancer, definitive diagnosis requires a tissue sample, or biopsy. A biopsy may be taken of a small area of the abnormality (an incisional biopsy), or the entire abnormal area may be removed at the time of biopsy (excisional biopsy). A skin biopsy might also be taken at the same time as the breast biopsy. Biopsy allows the pathologist (a physician with special training in the diagnosis of diseases based on tissue samples) to determine if cancer is present, and if so, what type of cancer. Biopsy also provides a tissue sample for further tests that are done (see below) to determine the best type of treatment. If a mass is not identified in the breast, a skin biopsy can sometimes be used to establish the diagnosis of inflammatory breast cancer. The diagnosis of inflammatory breast cancer can be challenging.

The diagnostic guidelines for inflammatory breast cancer require that the following factors are present:

  • A rapid onset of erythema (redness), edema (swelling), and a peau d'orange appearance and/or abnormal breast warmth, with or without a lump that can be felt
  • These symptoms have been present for less than six months.
  • The redness covers at least one-third of the breast.
  • A biopsy from the affected breast reveals invasive cancer.

Specialized testing on breast cancer samples

Certain laboratory tests are typically performed on all breast cancer tumor samples to help determine the optimum treatment. These include:

  • Hormone receptor status: Breast cancer tissue is tested for the presence of receptors for the hormones estrogen (estrogen receptor or ER) and progesterone (progesterone receptor or PR). If these receptors are present, the tumor is referred to as hormone receptor-positive. This means that hormone-directed therapies may be effective in stopping tumor growth.
  • HER2: Another test performed on breast cancer tissue measures the overexpression of a protein called HER2. If a tumor is HER2-positive (HER-2+), drugs that target this specific protein may be given. About 15% of women have so-called triple-negative breast cancers. This means that they do not express any of these tumor markers (ER, PR, or HER2). As mentioned before, inflammatory breast cancers often do not express the hormone receptors ER and PR. Newer laboratory tests may be useful for some types of tumors to help determine the prognosis and treatment plan. These include, for example, studies of gene expression in the particular tumor or tests to look for the presence of circulating tumor cells or tumor DNA.

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What are the stages of inflammatory breast cancer?

Staging of a cancer refers to the determination of how far the tumor has spread at the time of diagnosis. Staging is determined by a variety of methods including results from surgical procedures, lymph node biopsy, and imaging tests. Staging is important because it aids in developing a treatment plan.

Cancer in situ (DCIS) is referred to as stage 0, because the tumor cells are located in the breast ducts and have not invaded the surrounding tissue. Invasive breast cancers are staged along a scale of I to IV, with stage I being the earliest stage and stage IV representing tumors that have metastasized to distant organs like the bones, lungs, or brain. Because inflammatory breast cancers have already spread into the lymphatic vessels and cause symptoms related to this presence in the lymphatic system, all inflammatory breast cancers are stage III or stage IV at diagnosis.

Are there home remedies for inflammatory breast cancer?

Although home remedies are not appropriate for the treatment of inflammatory breast cancer, it is important to maintain a healthy lifestyle while undergoing medical treatment. This includes eating a healthy diet, smoking cessation, exercise, and getting adequate rest and sleep.

What is the treatment for inflammatory breast cancer?

Treatment for inflammatory breast cancer typically involves chemotherapy, surgery, and radiation. In some cases, additional targeted therapies are given. The term neoadjuvant refers to therapies that are given prior to surgery, while adjuvant refers to treatments given after surgery.

Neoadjuvant chemotherapy refers to chemotherapy medications that are given prior to surgery to shrink the tumor. In patients with inflammatory breast cancer, this is often done so that the tumor is smaller and easier to remove at surgery. A typical course of neoadjuvant chemotherapy would involve at least six cycles of chemotherapy over four to six months. The chemotherapy regiment usually involves taxane and anthracycline drugs.

If the tumor cells express the HER2 protein (see above), targeted therapies such as trastuzumab (Herceptin) can also be given as a neoadjuvant (prior to surgery) therapy and continued after surgery (adjuvant therapy). Inflammatory breast cancers are often positive for the HER2 protein, so the tumors can be responsive to treatments that target this protein. In addition to trastuzumab, other drugs that target HER2 activity are available.

It is less common for inflammatory breast cancers to express hormone (ER or PR) receptors, but if the tumor is hormone receptor-positive, hormone therapies that target estrogen can be effective. Tamoxifen is a drug that interferes with the activity of estrogen in the body and is a common hormone therapy drug. It can be given to both premenopausal and postmenopausal women. In postmenopausal women, drugs known as aromatase inhibitors are also used as hormone therapy. Examples of aromatase inhibitors include anastrozole (Arimidex), letrozole (Femara), and exemestane (Aromasin).

Surgery for inflammatory breast cancer usually involves a modified radical mastectomy with removal of lymph nodes, since the tumor has spread into the lymphatic system. Following surgery, radiation therapy is typically administered to the chest wall. This treatment uses radiation to destroy remaining cancer cells. The most common type of radiation therapy is given in a clinic, usually five days a week for several weeks.

Adjuvant therapy after surgery can include hormone therapy, chemotherapy, targeted therapy, or a combination of these methods described above.

Clinical trials, in which new drugs, new therapies, or combination of drugs and therapies are tested, may be available for some patients with inflammatory breast cancer and offer an additional treatment option. Anyone interested in taking part in a clinical trial should discuss this possibility doctor. Information about clinical trials is available from the National Cancer Institute's Cancer Information Service at 1-800-4-CANCER and at http://www.cancer.gov/clinicaltrials.

What are the survival rates and prognosis for inflammatory breast cancer?

The prognosis, or survival rates, for breast cancer depend upon the extent to which the cancer has spread and the treatment received. Because inflammatory breast cancer is diagnosed when the cancer has already spread to the lymphatic system, survival rates for this type of breast cancer are not as favorable as for other types of breast cancer. Still, it is important to remember that statistics about breast cancer are based on studies of patients who were diagnosed years ago, and since therapies are constantly improving, current survival rates may be even higher.

In terms of inflammatory breast cancer, some factors are associated with a better prognosis (more favorable outcome):

  • Stage: Stage III tumors have a better prognosis than stage IV tumors.
  • Grade: Higher-grade tumors (grade refers to the extent to which the cells resemble normal breast cells under a microscope; the higher the grade, the more abnormal the cells) tend to have a worse prognosis.
  • Studies have shown that African American women with inflammatory breast cancer have a worse prognosis than women of other racial groups.
  • Inflammatory breast cancers that are estrogen receptor-positive tend to have a better prognosis than those that are estrogen receptor-negative.
  • Women who undergo multimodal therapy (a combination of chemotherapy, surgery, and radiation therapy) have a better prognosis than women who do not have this type of therapy.

According the NCI's Surveillance, Epidemiology, and End Results (SEER) program, the five-year median survival for women with inflammatory breast cancer (from 1990 through 2008) was 57 months for stage III disease and 21 months for stage IV disease.

Is it possible to prevent inflammatory breast cancer?

There is no guaranteed way to prevent any type of breast cancer. Modifying the risk factors that can be altered (increase exercise, keep a good body weight, etc.) can help in decreasing the risk of getting breast and other cancers. Some women who are at increased risk of breast cancers may be given medications to decrease their risk or even undergo preventive mastectomy if they are at very high risk for breast cancer. None of these measures, though, are 100% effective in preventing breast cancer.

REFERENCES:

American Cancer Society. "Inflammatory breast cancer." <http://www.cancer.org/cancer/breastcancer/moreinformation/inflammatorybreastcancer/inflammatory-breast-cancer-toc>.

United States. National Cancer Institute. "Inflammatory breast cancer." Jan. 6, 2016. <http://www.cancer.gov/cancertopics/factsheet/Sites-Types/IBC>.

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Reviewed on 8/24/2016
References
REFERENCES:

American Cancer Society. "Inflammatory breast cancer." <http://www.cancer.org/cancer/breastcancer/moreinformation/inflammatorybreastcancer/inflammatory-breast-cancer-toc>.

United States. National Cancer Institute. "Inflammatory breast cancer." Jan. 6, 2016. <http://www.cancer.gov/cancertopics/factsheet/Sites-Types/IBC>.

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