Inflammatory breast cancer is a very aggressive but rare form of breast cancer. It constitutes approximately 1 to 5 percent of all breast cancer cases. In this article, we will discuss what exactly inflammatory breast cancer is, its manifestations including signs and symptoms, how it is diagnosed, its staging, the various lines of treatment available for it, the prognosis, survival rates, and projected life expectancy for the patients who have it.
What is breast cancer?
Breast cancer is a disease in which the cells that normally form the breast tissues multiply and grow uncontrollably, often due to a mutation in a certain gene, until the abnormal cells form a mass that puts pressure on the nearby breast structures. As with all kinds of cancer, breast cancer is classified into benign and malignant masses. While benign tumors may sound harmless, they can still deform the breast and cause much discomfort. In actuality, the word “benign” simply means that this type of breast cancer will stay at the one location where it originated, and will not spread to the rest of the body. On the other hand, malignant breast cancer has the ability to “metastasize” or spread to areas of the body outside the breast, most commonly including the liver, brain, lungs, and bone. Not only does this cause pain and harm in multiple body sites, it also means that removing the original tumor through surgery will not cure the disease, since the other tumors can keep growing and spreading. Breast cancer occurs almost exclusively in women, but may very rarely arise in male patients.
That’s not the only way to classify the different types of breast cancer. After taking out a piece of the tumor, it is put on a tiny glass slide and viewed using a high-powered microscope. Depending on what the pathologist sees through the lens, he/she may assign the tumor a certain “grade”. Grading tumors is based on how abnormal the cells look. If they look just slightly off, the tumor is given a low grade, which usually translates into a good prognosis. If the cancer cells have very dark and large nuclei, look like they’ve been placed haphazardly, and show evidence that they are capable of an abnormally high rate of replication, the tumor is more likely to be labelled a high grade tumor, which usually means a less promising prognosis for the patient.
Symptoms of inflammatory breast cancer
Conventionally, breast cancer first manifests itself when the patient notices a lump or mass in her breast. After that, other symptoms may follow, such as breast pain or an abnormal discharge from the affected nipple. Not so with inflammatory breast cancer. As the name suggests, it causes strange, atypical early signs and symptoms that are normally attributed to inflammation rather than cancer. Thus, it is uncommon to find a lump affecting the breast of a patient if she has inflammatory breast cancer. Instead, she may complain of one of the following symptoms, which usually develop surprisingly rapidly (within weeks), unlike those of traditional breast cancer:
- Appearance of a rash or redness.
- Breast pain or tenderness.
- A bruise that does not seem to heal.
- Rapid swelling.
- Discharge from the nipple.
- A turned in or retracted nipple.
- An area of skin that looks similar in appearance to an orange peel.
- An itchy breast.
- Enlargement of the lymph nodes present in the armpit or in the neck.
- Abnormal warmth of the breast.
- In some cases, a lump may be present.
A patient who suffers from inflammatory breast cancer may not show all of these symptoms. There are even some cases that do not show any symptoms at all, which are said to have “occult” breast cancer.
Skin cells normally get rid of the leftovers and waste products of metabolism by sending them into the lymphatic channels, which effectively drain the skin, almost functioning like a miniature sewage system. In inflammatory breast cancer, these lymphatic channels become clogged by the tumor cells, and are unable to drain the breast, thus a rapid swelling of the breast is seen. However, this is not a uniform swelling. The skin of the breast is tethered to the collarbone and other tissues by a ligament called the Suspensory ligament of Cooper. For this reason, when a swelling of the skin of the breast happens: we see many tiny dimples throughout the swelling. This fact, in addition to the reddish appearance of the breast due to the inflammation, gives the breast skin an appearance uncannily similar to that of an orange peel. Thus, this appearance is termed “Peau d’orange”.
Another problem that can happen when inflammatory breast cancer cells enter the lymphatic system is that they can spread to other body parts. This can occur if the cells circulate in the blood as well. As mentioned above, the common sites of cancer spread are the lungs, the liver, the bones, and the brain. Depending on which place the breast cancer cells ultimately end up at, different manifestations can appear. For example, if inflammatory breast cancer cells spread to the lungs, the affected individual may find herself having difficulty breathing, and even coughing blood.
Inflammatory breast cancer vs mastitis
Inflammation is a complicated process that the body undertakes in order to fight off infection and remove any dead cells or microscopic debris. It involves widening of blood vessels or “Vasodilation”, the transport of various immune cells to the site of the inflammatory response, the use of biological markers and chemical mediators to call in more reinforcements, and the destruction of the targeted cells or pathogens.
The inflammatory response is intended to be a useful weapon for the body, but things can go wrong quickly if the inflammatory cells are not actually needed or if the inflammation overstays its welcome. In these cases, inflammatory is harmful and damaging to the body and should be stopped as soon as possible.
One example of what can go wrong with inflammation is a condition known as mastitis. It refers to an inflammation of the breast tissue. Bacteria can enter the breast through a cracked nipple, causing an inflammatory response in the breast. Breast inflammation can also occur if the milk ducts of the mammary gland become obstructed for any reason and the milk becomes trapped inside. Thus, mastitis commonly appears in women who are pregnant or breastfeeding, where its incidence is highest in the first 6 to 12 weeks after giving birth. It is also possible for non-lactating women to develop mastitis, albeit at a lower rate.
In general, there are some cardinal signs that always appear when inflammation is present. These are pain, swelling, redness, heat, and loss of function. The problem we are faced with here is that while, naturally, mastitis exhibits these criteria since it is a type of inflammation, inflammatory breast cancer also produces similar signs and symptoms. The first is a benign disease that can go away on its own, while the second is a grave condition that has a high mortality rate and is rather difficult to treat. So, how do we differentiate between mastitis and inflammatory breast cancer?
For starters, mastitis tends to occur more commonly in younger women who are pregnant or breastfeeding, while inflammatory breast cancer is more likely to afflict older females who have entered the period of menopause.
Another important differentiator is that when a patient with what seems to be an inflammation of the breast is given antibiotics for a minimum of one week, the signs and symptoms will improve if the disease is mastitis, while a case of inflammatory breast cancer would continue to experience the disease’s manifestations.
Staging of inflammatory breast cancer
The staging of a cancer is a method used to assess the severity of the disease and the potential danger it poses. It depends on the size of the tumor, whether or not it has spread to the regional lymph nodes, and if it has spread to other parts of the body. The well-known term “Stage four cancer” refers to a malignancy that has spread to one or more body sites other than its primary place of origin. Cancer stages are usually divided into stage I, II, III, and IV. In between the main stages, there are smaller, more detailed stages like 1B or IIIC.
Inflammatory breast cancer does not only affect the breast tissue, but also invades the skin. For this reason, the first rung in inflammatory breast cancer staging is not stage I but stage IIIB. This is one of the reasons why inflammatory breast cancer often has a less promising prognosis than conventional breast cancer.
If the lymph nodes present around the collarbone (clavicle) or those in the chest are also affected by the breast cancer cells, it is a stage IIIC breast cancer. And as noted above, any breast cancer of any size that has metastasized beyond its original location is termed a stage IV cancer.
Diagnosis of inflammatory breast cancer
Traditionally, diagnosis of any tumor begins with one or more radiological studies. These include the chest x-ray, where waves of electromagnetic radiation penetrate the chest and thus create an image on a radiographic film delineating the various structures, normal and abnormal, present in the chest.
Another method of radiological examination is a CT (Computed Tomography) scan, where the chest is photographed at multiple levels or “slices”, so that the radiologist can visualize all structures present at every level. This is sometimes combined with a PET (Positron emission tomography) scan to detect cancer deposits in the nearby lymph nodes.
The fourth modality is an MRI (Magnetic resonance imaging) scan, which is the most accurate, safest but most expensive one. It uses powerful magnets to create a superbly detailed image of the examined body part.
A special type of radiography, created specifically for diseases of the breast, is the mammogram. In this method, the breast in question is isolated and compressed between two curved plastic plates in order to flatten it and thin out the breast tissue, allowing for more effective penetration by the x-rays. The radiation beams are fired from multiple angles as well to create a more accurate and comprehensive image. Mammograms are safe, cheap, effective, and available everywhere. They do sometimes cause a bit of discomfort when the breast is being compressed, but this is usually mild and will often disappear quickly. A more advanced technique in mammography is the 3D mammogram, where the breast is not just photographed from two angles. Instead, the camera used revolves around the diseased breast, shooting electromagnetic radiation waves from all directions. This results in a slightly higher dose of radiation being received by the patient, but it usually isn’t an issue and the resulting image has a very high level of detail.
A breast ultrasound may be performed as well, which uses a probe that sends out ultrasonic waves to check for any abnormal structures.
However, inflammatory breast cancer is particularly tricky to diagnose with radiography alone. The methods listed above may suggest the disease by showing suspicious signs, but the only way to get a sure diagnosis is through a biopsy.
A tissue biopsy is a procedure where the surgeon extracts a part or all of a suspected tumor. This is often done using a needle and may be combined with an ultrasound or a CT scan for added accuracy. In some cases, however, the tumor may need an incision in order to get a biopsy. After a suitable amount of tissue is obtained, it is stained and put on a glass slide for microscopic examination. This will determine the histological subtype of the breast cancer as well as its grade, which describes how abnormal the cells look. Both factors are very important in the line(s) of treatment used, the prognosis, the survival rate, and the estimated life expectancy.
Treatment of inflammatory breast cancer
The treatment lines used for a case of breast cancer can be divided into surgery, chemotherapy, radiotherapy, immunotherapy, and hormonal therapy.
Although inflammatory breast cancer is a very aggressive disease, and its prognosis is less favourable than the other types of breast cancer, there is a treatment regimen that has been associated with the best possible outcome. This consists of a course of chemotherapy, followed by a surgical procedure, then postoperative radiotherapy.
Chemotherapy is the use of very strong drugs that are given either orally or by injection, which move through the circulatory system until they reach the tumor, and they then attack and destroy the tumor cells. These drugs have a predilection for tumor cells, but are so powerful that they usually cause some undesirable side effects, like nausea and vomiting. A patient may be prescribed one or more of these drugs depending on what the physician decides is the best regimen to maximize the effectiveness and minimize the side effects.
Surgery for inflammatory breast cancer usually follows chemotherapy, where the drugs reduce the size of the tumor, allowing for an easier operation. The goal of any cancer surgery is to remove as much tumor tissue as possible, while preserving the largest amount of normal tissues possible.
The surgical procedure used is often a modified radical mastectomy, where the entire breast is removed. In this operation, the patient is put to sleep using general anesthesia. The patient lies on her back, with her arms forming a right angle with the body. The boundaries and landmarks of the breast are delineated using a marker. The incision is then made in such a way so as to allow the surgeon to close the wound after the operation with minimal difficulty. An important step in this surgery is the dissection of the lymph nodes present under the arm. These lymph nodes are commonly hiding cancer cells, which are often responsible for inflammatory breast cancer recurrence if only the breast is removed. All in all the breast tissue, skin, areola, nipple, and the axillary lymph nodes are removed, while the large pectoralis major muscle is preserved. This is why the operation is called a “modified” radical mastectomy. A radical mastectomy involves removing the pectoralis major chest muscle as well. The patient is usually discharged the next day, and encouraged to move her arm since lymph node removal leaves the arm somewhat sore and stiff. Some arm exercises may be arranged for the patient. The surgeon will usually place small plastic tubes called drains to remove any excess fluid present in the surgical site, which usually remain in place for one or two weeks.
Less invasive surgeries such as removing the tumor alone have not been proven to be effective.
After the operation has been carried out and the breast removed, the patient may undergo breast reconstruction surgery later or in the same operation. This restores the cosmetic appearance of the breast and improves the patient’s psychological well-being after the mastectomy. One method to reconstruct the breast is to use the body’s own tissues, while preventing any loss of function. The surgeon may use a nearby body structure and mobilize it to occupy the area of the breast, while still being connected to its original blood supply. An example of this is to use a large back muscle called the Latissimus Dorsi, which has enough volume to mimic the normal appearance of the breast while minimising the loss of function. The second option is for the surgeon to use a far away piece of tissue whose original blood supply cannot be maintained, so the physician must cut the original arteries and veins, then use a type of microscope to connect the tissues to the arteries and veins present in the breast area, which requires great expertise and skill. The second method used for breast reconstruction is to use an artificial implant, which is the more common procedure.
Radiotherapy is often used after the surgical removal of the breast, and aims to kill any remaining tumor cells and prevent future cancer recurrence. This line of treatment uses powerful radiation waves aimed at the tumor, which are strong enough to destroy cancer cells.
Immunotherapy is the use of certain drugs that stimulate the body’s immune cells in order to attack the cancer cells with increased effectiveness.
Hormonal therapy, while a great treatment modality for most types of breast cancer, is unfortunately ineffective in managing inflammatory breast cancer. Normally, the female gonads (the ovaries) produce estrogen and progesterone, the two female sex hormones, from the onset of puberty till the onset of menopause. They help give rise to the female sexual characteristics such as a high pitched voice and a feminine body shape. However, some types of breast cancer, termed hormone receptor positive breast cancers, have the ability to utilize these two hormones for growth and proliferation through tiny receptors that bind to the estrogen and progesterone molecules. This feature can actually be used against the tumors by using drugs that mimic the two female sex hormones, which bind to and block the hormone receptors present on the surface of the tumor cells, preventing the cancers from growing. Most cases of inflammatory breast cancer are found to be of a variant called “Triple negative breast cancer”, which does not possess estrogen or progesterone receptors, and therefore cannot be attacked using hormonal therapy. This characteristic also does not bode well for the patient’s prognosis, since triple negative breast cancer generally has a higher mortality rate than hormone positive breast cancer.
If the tumor is found to be particularly resistant to standard treatment modalities, the patient may choose to enter into a clinical trial. Clinical trials are done to test out new lines of treatment that have not yet been authorized to use on a mass scale. These trials are performed to check the effectiveness and safety of new experimental drugs, and are the best way to bring novel, lifesaving new treatments to patients worldwide.
Prognosis and survival rates for inflammatory breast cancer
Breast cancer, in general, causes one in eight women to lose their life. Inflammatory breast cancer, while it is rare at 1 to 5 out of every 100 cases of breast cancer, has a higher mortality rate than other types of the disease. One third of women who are diagnosed with inflammatory breast cancer are first diagnosed after the tumor has metastasized.
The prognosis of a cancer is measured using the percentage of patients who survive for at least five years after the initial diagnosis, thus the metric we use is called the 5-year survival rate.
It was found that the 5-year survival rate for inflammatory breast cancer in general is about 39%
If it has been confirmed that the tumor has spread to the nearby lymph nodes, the 5-year survival rate is 52%
If the cancer has been found to have spread to other body parts, then the 5-year survival rate falls to 18%