What is Inflammatory Breast Cancer?
Inflammatory breast cancer (IBC) is a rare but severe form of advanced breast cancer that makes up 2% to 4% of all breast cancer cases in the US. Despite being less common, it causes about 7% of deaths due to breast cancer. This cancer typically leads to the skin of the breast becoming hard and looking like a skin infection, even though no lump can usually be felt underneath.
The American Joint Committee on Cancer (AJCC) has clear guidelines for recognizing IBC—it’s a unique type of cancer that causes redness and swelling covering at least a third of the breast. It can even spread to cover the entire breast, the other breast, and extend to the chest area, arms, and neck.
To diagnose IBC, doctors use the TNM system developed by the AJCC, where it’s classified as T4d. Your doctor will be looking for several signs to diagnose this kind of cancer, including:
- A quick onset of redness, swelling and/or a condition known as peau d’orange (skin that looks like an orange peel), a warm feeling in the breast, which may or may not have a lump that can be felt.
- A history of these symptoms for six months or less.
- Redness covering at least a third of the breast.
- A confirmed diagnosis through a laboratory test showing invasive cancer cells.
It’s also essential to distinguish between primary and secondary inflammatory breast cancer. Primary inflammatory breast cancer occurs when cancer develops in a breast that was previously healthy. Secondary inflammatory breast cancer refers to when a breast with other types of invasive cancer or a breast that had a previous surgery starts showing the signs of inflammation associated with IBC.
What Causes Inflammatory Breast Cancer?
Having a high body mass index, or BMI, is a risk factor in developing inflammatory breast cancer. This means that if you have a higher BMI, you’re more likely to get this type of cancer.
There are a few other areas that are being researched to see if they could be risk factors too. These include getting a viral infection or having chronic inflammation – which is when part of your body becomes inflamed for a long time. Other potential factors being looked at include: having a child at a younger age, smoking, and breastfeeding.
Interestingly, having certain changes in your genes (known as inherited genetic mutations) and having a family history of the disease have not been shown to increase your chances of getting inflammatory breast cancer.
Risk Factors and Frequency for Inflammatory Breast Cancer
The National Cancer Institute’s surveillance program shows that the occurrence of Inflammatory Breast Cancer (IBC) increased in the 1990s, with only a little improvement in survival rates. Notably, age at diagnosis, incidence rates, and survival outcomes varied considerably among different racial groups. The occurrence of IBC remained fairly stable from 1990 to 2002.
IBC is recognized as the most aggressive type of breast cancer. On average, women survive less than 4 years even with various treatment options. Improvement in chemotherapy management has led to slightly longer survival rates in recent years. Notably, both ethnicity and socio-economic status have been found to significantly influence the risk of dying from breast cancer and the age of diagnosis respectively.
- Inflammatory breast cancer rate increased in the 1990s, then remained stable from 1990 to 2002.
- It’s the most aggressive type of breast cancer, with an average survival length of less than 4 years.
- Chemotherapy management improvements have slightly increased survival rates recently.
- Both ethnicity and socio-economic status can influence the risk and diagnosis age.
- IBC is more common in low-income countries than in Western countries, with potential causes including limited access to screening, late diagnosis, and scarce medical services.
- IBC incidence is higher among Black women than in White women.
Signs and Symptoms of Inflammatory Breast Cancer
Inflammatory breast cancer is a severe condition where individuals typically experience symptoms like pain, a quickly growing lump in the breast, itching, and an enlarged breast. Almost every patient has lymph node involvement when they first notice these symptoms, and about one third might even have the cancer spread to other parts of the body. This could lead to symptoms like swollen lymph nodes and pain in different areas, depending on how far the disease has spread.
These symptoms can develop quickly, over weeks to months. Sadly, most people are first treated with antibiotics, thinking that they have an infection or abscess in the breast. If there is no improvement with these treatments, they then seek help from experts. About 10% of these cases are discovered through routine breast cancer screenings known as mammograms.
During a physical examination, the breast might look distorted or bigger than usual. The skin may resemble the skin of an orange, feeling warm and thick. There may also be issues with the nipple like redness, flattening, blisters, or the nipple being pulled inward. A lump that you can feel might be there, but not always. It’s important for healthcare providers to examine both breasts and the armpit area thoroughly for signs of lymph nodes being affected.
Testing for Inflammatory Breast Cancer
The diagnosis of inflammatory breast cancer (IBC) is made through a patient’s symptoms and a biopsy confirming breast cancer. This kind of cancer needs to be strongly considered if a patient has fast-developing inflammation in their breast that is not getting better with antibiotics. If a doctor suspects inflammatory breast cancer, breast imaging and a biopsy should take place.
A mammogram, or breast x-ray, is usually the first imaging test a doctor will request when checking for IBC. A diagnostic mammogram will be performed on the breast showing symptoms, and a screening mammogram will be conducted on the other side to compare. If a doctor suspects cancer, an ultrasound of both the breasts and any nearby lymph nodes should also be conducted.
The mammogram could show several key signs of IBC, which may include a large mass, a big area of calcification, or distortions in the breast tissue accompanied by skin thickening. Note that both mastitis, which is a breast tissue infection, and IBC without an identifiable mass may appear similar on a mammogram. If antibiotics don’t improve the condition within about a week, a biopsy should be conducted to check for cancer.
Ultrasound imaging might show several things, such as a thickening of the skin, fluid between tissue layers, and disruption of normal breast tissue. This method can help better understand the nature of the masses, guide a doctor during a needle biopsy and lymph node biopsies.
Techniques like mammography and ultrasound often don’t provide clear images when checking for IBC because of the considerable swelling and redness which are classic symptoms of this disease. The necessary compression for a good mammogram may also cause discomfort for patients with IBC. The combination of inadequate tissue compression, widespread skin and breast tissue thickening, and swelling can hide masses on a mammogram. Similarly, using ultrasound to identify a primary breast lesion and guide a needle biopsy may be more difficult because of the widespread swelling and distortions in the breast tissue structure.
However, magnetic resonance imaging (MRI) might be able to show small, merging masses and overall skin thickening––which are both key signs of IBC. This can aid in better detection of breast cancer and better understanding of how far the disease has spread compared to just using a mammogram. In tougher cases, an MRI may assist in guiding the biopsy. It can also be used to monitor how well a patient responds to chemotherapy. MRI is generally the most reliable imaging method for spotting primary breast anomalies in people with IBC. An ultrasound can be useful in identifying disease in regional lymph nodes.
Positron emission tomography/computed tomography (PET/CT) scan with fluorodeoxyglucose (FDG), may provide additional information about lymph nodes or far-off metastases (the spread of cancer cells from the primary site to other areas of the body) when initially evaluating a person with IBC. A PET scan can be used to stage the cancer and help tell apart curable and incurable states as well as assess the patient’s response to therapy.
In line with other invasive breast cancers, all IBC tumors should be tested for hormone receptors (proteins that receive signals from hormones and cause cellular changes) and the human epidermal growth factor receptor 2 (HER2, a protein aiding cell growth and division).
Treatment Options for Inflammatory Breast Cancer
Both the National Comprehensive Cancer Network and international guidelines for treating inflammatory breast cancer recommend intensive treatment combining multiple methods: medication, surgery, and radiation. A team of health professionals is essential in properly managing and treating inflammatory breast cancer.
Inflammatory breast cancer that hasn’t spread to other parts of the body is treated similarly to other non-invasive forms of advanced breast cancer. However, a key difference is that lymph node biopsy and breast-preserving therapy aren’t typically chosen for inflammatory breast cancer, even if the cancer responds well to initial treatment. Since this type of cancer is rare, eligible patients are often enrolled in clinical trials.
The standard treatment includes initial chemotherapy, followed by therapy targeting the local area where the cancer exists. Patients who respond partially to the initial chemotherapy usually undergo a specific type of breast removal surgery, along with treatment to the underarm area, and post-surgery radiation. Patients unable to have surgery are given radiation therapy. The success of the treatment is measured by the cancer’s response to it. Patients whose cancer is completely eliminated by this treatment usually have a better outcome than those whose aren’t.
Chemotherapy involving specific types of drugs is usually the first course of treatment. For inflammatory breast cancer with high levels of a certain protein (HER-2), additional medications may be used in combination with chemotherapy. Hormone therapy may also be used, depending on the patient’s hormonal status and menopausal state.
Inflammatory breast cancer has a poor prognosis and a high likelihood of recurring early, so immediate reconstruction of the breast after surgery is typically avoided. Recent evidence suggests that outcomes have improved with chemotherapy, followed by local, regional treatment.
In some cases, patients already have advanced inflammatory breast cancer at the time of diagnosis. The treatment for this is primary therapy involving medication to achieve the best response. Patients who respond significantly to this treatment should also receive intense therapy targeting the area where the cancer exists. However, there’s a debate about the impact of surgery and radiation therapy on the overall survival of these patients. The goal of treating advanced inflammatory breast cancer is to prolong survival, alleviate symptoms, and improve the quality of life, even considering the potential side effects of the treatment.
What else can Inflammatory Breast Cancer be?
There are several conditions that can be mistaken for inflammatory breast cancer. It’s crucial to be cautious because misdiagnosing can delay the proper diagnosis and treatment. Some conditions that can mimic inflammatory breast cancer include:
- Ductal ectasia: This is a non-cancerous condition usually found in middle-aged women. It happens when skin debris collects and causes the breast ducts to swell. It usually gets better without treatment.
- Infectious mastitis and breast abscess: These are infections of the breast that can occur in a nursing mother. They often come with fever and an increase in white blood cells and typically get better with antibiotics. They can appear similar to inflammatory breast cancer in medical examinations and on mammograms.
- Noninflammatory breast cancer: Sometimes it can be tough to tell inflammatory breast cancer from a type of advanced breast cancer that affects the skin. However, signs like skin dimpling, nipple pulling inward, skin bumps, skin swelling, or sores on their own, without other diagnostic criteria, don’t indicate inflammatory breast cancer.
Other serious illnesses, like breast lymphoma or leukemia, can also appear similar to inflammatory breast cancer. However, these illnesses can be identified accurately with a biopsy and by examining tissue under a microscope.
What to expect with Inflammatory Breast Cancer
Inflammatory breast cancer is linked with a very poor outlook and a high chance of the cancer returning early. When comparing the same stage of locally advanced breast cancer, inflammatory breast cancer tends to have a less favorable outlook.
Several factors can indicate a worse outlook, including negative receptor status (this means the cancer cells don’t have certain proteins that respond to hormones), involvement of four or more lymph nodes at the time of diagnosis, and not responding to the first round of chemotherapy (known as neoadjuvant chemotherapy).
However, with the introduction of three-pronged treatments, the current 5-year survival rates range from 30% to 70%. This means that, on average, about 30%-70% of people with this condition will still be alive five years after diagnosis.
Possible Complications When Diagnosed with Inflammatory Breast Cancer
Inflammatory breast cancer can lead to complications due to the spread of the disease locally and to different parts of the body. These complications can include intense pain, such as pain in the bones and nerves. If the cancer spreads to the bone, it can lead to a condition called hypercalcemia, which can cause kidney stones and create neurological issues such as memory problems, confusion, and even cause comas and irregular heartbeats.
In about half of the cases, the cancer spreads to the liver, causing discomfort in the abdomen, feeling sick, throwing up, and jaundice (a yellowing of the skin and eyes). As the cancer advances, it can also cause loss of appetite and weight. If the cancer spreads to the brain, it may affect a person’s vision and behavior, lead to memory problems, and cause headaches that get progressively worse.
Treatment for breast cancer can also have side effects. Chemotherapy can cause mouth sores, feeling sick, and diarrhea. Surgery carries potential risks including the risk of the surgery itself, the risk of anesthesia, infection, poor healing, and cosmetic issues. Lymphedema, a condition that causes swelling in the arms, is a significant complication that can occur after surgical removal of the lymph nodes in the armpit. Radiation therapy can cause severe pain and scarring.
Dealing with inflammatory breast cancer can also have a major impact on a person’s personal life, causing significant stress, and can have economic impacts as well.
Potential Complications:
- Intense pain
- Hypercalcemia
- Neurological issues
- Liver lesions
- Abdominal discomfort
- Nausea
- Vomiting
- Jaundice
- Weight loss
- Brain complications
- Mouth sores (due to chemotherapy)
- Diarrhea (due to chemotherapy)
- Risks associated with surgery
- Lymphedema
- Pain and scarring (due to radiation therapy)
- Social and economic stress
Preventing Inflammatory Breast Cancer
Breast cancer is the most frequently diagnosed cancer amongst women in the United States and is also the second leading cause of cancer-related deaths among women. Detecting and treating breast cancer early drastically improves a woman’s chance of survival. This is why self-examinations and regular screenings, such as mammograms, play a key role in early detection of the disease.
It’s essential to respect and take into account a patient’s cultural beliefs and practices when assessing their health and planning their treatment. We strongly encourage people to participate actively in raising awareness about breast cancer and its treatments, alongside the local community, healthcare professionals, and relevant organizations. This involvement can ensure that everyone is educated about the disease, which can significantly help in dealing with it effectively.
