Inflammatory Carcinoma of Breast Stage IV

Inflammatory breast cancer at stage IV is a rare and highly aggressive form of breast cancer that has spread beyond the breast to other parts of the body. Understanding this condition, its symptoms, and treatment options is essential for patients and their families navigating this challenging diagnosis.

Table of contents

• What is Inflammatory Breast Cancer?
• Understanding Stage IV Disease
• Signs and Symptoms
• How is it Diagnosed?
• Treatment Approaches
• Outlook and Prognosis

What is Inflammatory Breast Cancer?

Inflammatory breast cancer (IBC) is a rare but aggressive type of breast cancer that accounts for about 1% to 5% of all breast cancer cases in the United States^[7]. This disease is called “inflammatory” because the breast often looks red and inflamed, but it is not caused by an infection^[7].

IBC happens when cancerous cells block lymphatic vessels (small channels that drain fluid from tissues) in one of your breasts. The blocked vessels cause inflammation, making your breast look swollen or discolored^[2]. Unlike other types of breast cancer, IBC typically does not cause breast lumps^[2].

Most inflammatory breast cancers are invasive ductal carcinomas, which means they began in the milk ducts^[7]. This type of cancer can spread rapidly from the lymphatic vessels in your breast to nearby breast tissue and lymph nodes or to other areas of your body^[2].

Women with inflammatory breast cancer tend to be diagnosed at a slightly younger age than women with other breast cancers^[7]. Black and African American women and women who have obesity are more likely to be diagnosed with inflammatory breast cancer^[7].

Understanding Stage IV Disease

According to the American Joint Committee on Cancer staging guidelines, inflammatory carcinoma is classified as T4d, which makes all patients with inflammatory carcinoma stage IIIB, IIIC, or IV depending on the nodal status and presence of distant metastases (cancer that has spread to other parts of the body)^[5].

Stage IV inflammatory breast cancer means the cancer has spread beyond the breast to other parts of the body. The most common places where breast cancer spreads include the bones, liver, lungs, and brain^[13]. However, breast cancer may also spread to other organs.

When inflammatory breast cancer reaches stage IV, it is often because the cancer can spread very quickly. Inflammatory breast cancer can spread (metastasize) rapidly, moving to other parts of your body like your brain, lungs, or liver^[2]. Often, the cancer is spreading by the time most women receive their diagnosis^[2].

In general, women with inflammatory breast cancer present at a younger age, are more likely to have metastatic disease at diagnosis, and have shorter survival than women with non-inflammatory breast cancer^[5].

Signs and Symptoms

The warning signs of inflammatory breast cancer are different from other types of breast cancer and tend to arise quickly, within weeks or months^[7]. With other breast cancers, warning signs may not occur for years.

IBC does not cause breast lumps like other types of breast cancer. Instead, it causes noticeable symptoms that develop rapidly and don’t go away^[2]:

• Swelling or enlargement of the breast, so that one breast looks bigger than the other
• Redness of the breast (may also appear as a pinkish or purplish tone)
• Breast dimpling or puckering
• Skin that looks and feels like an orange peel (called peau d’orange)
• Bruising or rash that spreads over one-third of your breast
• An inverted or retracted nipple (a nipple that points inward or is pulled in)
• Pain, swelling, itchiness, firmness, or tenderness in the breast
• Swollen lymph nodes near your collarbone or under your arm
• Warmth or burning sensation in the breast

In women of color with deeper skin tones, redness of the breast skin may be more difficult to see^[7]. Sometimes a lump can be felt, but it’s less common with inflammatory breast cancer than with other breast cancers.

If any of these changes last longer than a week, you should tell your healthcare provider^[7]. If you’re not comfortable with your healthcare provider’s recommendation, it’s always okay to get a second opinion.

How is it Diagnosed?

Inflammatory breast cancer is a clinical diagnosis, which means the diagnosis is based on your symptoms and a physical examination, along with test results^[6]. The process often starts with a discussion of your health history and an exam of the breast.

Physical Examination

Your healthcare professional will examine your breast to look for changes in skin color, swelling, and other signs of inflammatory breast cancer^[6]. They will ask about your symptoms, including when you first noticed changes in your breast^[2].

Imaging Tests

Your healthcare professional may recommend several imaging tests to look for signs of cancer in your breast^[6]:

• Mammogram (breast X-ray) to look for IBC signs, like thickening breast skin or unusual amounts of calcium. The mammographic appearance of inflammatory breast cancer differs from other breast tumors because less than half will show a discrete mass^[5].
• Breast ultrasound that may detect changes in your breast tissue
• MRI (magnetic resonance imaging) to look for an abnormal mass or other changes in your breast tissue, which may be recommended in certain situations^[2]

Biopsy

Your provider may refer you to a surgical oncologist for a breast biopsy. This is a procedure to remove a sample of tissue for testing in a lab^[6]. The tissue might be removed using a needle that is put through the skin and into the suspected cancer cells. A skin biopsy also may be helpful. This type of biopsy removes a sample of skin cells^[6].

A medical pathologist will test the sample for cancerous cells. If they find cancerous cells, your pathologist will check for receptors, which are special proteins that help cancerous cells grow^[2].

Tests to Determine Cancer Spread

If you’re diagnosed with inflammatory breast cancer, you may have other tests to see if the cancer has spread. These tests help your healthcare team find out the extent of your cancer, also called the stage^[6]. Imaging tests may include:

• MRI scan
• CT scan (computed tomography) to look for cancer in your soft tissues and bones
• Bone scan to look for cancer in your bones
• PET scan (positron emission tomography) that uses radioactive medicines to create images of your organs and tissues

Your healthcare team uses the cancer staging test results to help create your treatment plan^[6].

Treatment Approaches

Treatment for stage IV inflammatory breast cancer requires immediate attention. Historically, this disease was uniformly fatal, but with the advent of combined treatment approaches, the prognosis of these patients has improved^[5]. With current therapy, approximately one-third of women diagnosed with inflammatory breast cancer will become long-term survivors^[5].

The type of treatment you have depends on several factors, including whether your cancer cells have receptors for particular types of drugs, your general health, and whether you have any other medical conditions^[2]. Your medical team will discuss the pros and cons of the treatment options with you.

Drug Treatments Before Surgery

Drug treatment before surgery is called neoadjuvant therapy. You usually have chemotherapy, which helps to shrink the cancer^[2]. If your cancer cells have HER2 receptors, you might also have targeted cancer drugs such as trastuzumab (Herceptin)^[2].

Surgery

After chemotherapy with or without targeted cancer drugs, you usually have surgery. You are most likely to have your whole breast removed, which is called a mastectomy^[2]. After a mastectomy, you may be able to have a breast reconstruction. Your surgeon may also remove the lymph nodes under your armpit.

Radiation Therapy

After surgery, you have radiation therapy. Radiation therapy uses high-energy X-rays to kill cancer cells. This helps to stop the cancer from coming back^[2].

Hormone Therapy

You may take hormone therapy tablets for some years if your breast cancer has hormone receptors. The aim of hormone therapy is to lower the risk of the cancer coming back^[2].

Treatment for Stage IV Disease

When inflammatory breast cancer has spread to other parts of the body (stage IV), treatment focuses on managing the disease and maintaining quality of life. Your care team may use a carefully coordinated combination of treatments tailored to your specific situation.

Outlook and Prognosis

Inflammatory breast cancer is indeed an aggressive disease. In general, the outlook for this type of cancer is worse than for other types of breast cancer because it can develop quickly and may spread to other parts of the body^[22].

However, thanks to research, survival has improved significantly with existing treatments and combinations^[14]. The advent of combined treatment approaches has resulted in a marked improvement in prognosis^[5].

It’s important to know that while living with metastatic disease (stage IV cancer) has not been an easy situation for many patients, it is something that has gotten much easier over the years^[17]. Any sort of chronic illness comes with a huge level of anxiety and stress, and how you manage that is a big part of how you survive and what the quality of life is as you survive^[17].

More people are living with metastatic breast cancer than ever before. In 2017, researchers at the National Cancer Institute estimated that more than 150,000 women in the United States were living with metastatic breast cancer^[19].

Support from others with the same diagnosis can be invaluable. A good support group, online or in person, can help you feel less isolated and more hopeful^[19]. Group members may be able to share their coping strategies with you.