Overview
Inflammatory Breast Cancer (IBC) is a rare and aggressive form of cancer where the cancer cells block lymph vessels in the skin of the breast. This type of breast cancer is called “inflammatory” because the breast often looks swollen and red, or inflamed. Inflammatory breast cancer is rare, accounting to up to 5 percent of all breast cancers. Most inflammatory breast cancers are invasive ductal carcinomas, which means they developed from cells that line the milk ducts of the breast and then spread beyond the ducts.
IBC progresses rapidly, sometimes in a matter of weeks or months. At diagnosis, inflammatory breast cancer is either stage III or IV disease, depending on whether cancer cells have spread only to nearby lymph nodes or to other tissues as well.
Additionally, inflammatory breast cancer has the following features:
Comparatively, inflammatory breast cancer tends to be diagnosed at younger ages unlike other breast cancer
Inflammatory breast tumours are frequently hormone receptor negative. This means they cannot be treated with hormone therapies that interfere with the growth of cancer cells driven by oestrogen.
It is more common in obese women than in women with normal weight.
Like other types of breast cancer, inflammatory breast cancer can occur in men as well but usually at an older age than in women.
Symptoms
Symptoms of inflammatory breast cancer include:
Swelling (oedema) and redness (erythema) that affect a third or more of the breast. The skin of the breast may also appear pink, reddish purple, or bruised. Additionally, the skin may have ridges or appear pitted, like orange peel. These symptoms are caused by fluid build-up in the skin of the breast. This happens due to cancer cells blocking the lymph vessels in the skin thus preventing the normal flow of lymph. Sometimes the breast may contain a solid tumour that can be felt during a physical exam
Other symptoms of inflammatory breast cancers are:
A rapid increase in breast size
Sensations of heaviness, thickness, burning, or tenderness in the breast
A nipple that is flattened or inverted (facing inward).
Swollen lymph nodes may also be present under the arm, near the collarbone, or both.
Discoloration, giving the breast a red, purple, pink or bruised appearance
Unusual warmth of the affected breast
It is important to note that these symptoms may also be signs of other diseases or conditions, such as an infection, injury, or another type of breast cancer that is locally advanced. Therefore, anyone suffering with inflammatory breast cancer often have a delayed diagnosis of their disease.
Causes
The causes of inflammatory breast cancer are unclear. However, it is known that inflammatory breast cancer begins with an abnormal cell in one of the breast’s ducts. Mutations within the abnormal cell’s DNA makes it grow and divide rapidly. The abnormal cells invade and block the lymphatic vessels in the skin of the breast. This blockage causes the red veins, swollen and dimpled skin, which are classic signs of inflammatory breast cancer. The factors that increase the risk of inflammatory breast cancer are:
Being a woman: Women are more likely to be diagnosed with inflammatory breast cancer than are men.
People of darker complexion have a higher risk of inflammatory breast cancer than people with fairer complexion.
Being obese have a greater risk of inflammatory breast cancer compared with those of normal weight.
Diagnosis
Inflammatory breast cancer is often difficult to diagnose. There are usually no lumps that can be felt during a physical exam or seen in a screening mammogram. Most women diagnosed with inflammatory breast cancer have dense breast tissue, which makes cancer detection in a screening mammogram difficult. Also, inflammatory breast cancer being aggressive, can arise between scheduled screening mammograms and progress rapidly. The symptoms could also be mistaken for those of mastitis, which is an infection of the breast, or other forms of locally advanced breast cancer.
To aid swift diagnosis and to choose the optimal course of treatment, there are guidelines on how doctors can diagnose and stage inflammatory breast cancer correctly:
A rapid onset of erythema (redness), oedema (swelling), and a peau d’orange appearance (ridged or pitted skin, like orange peel) and/or abnormal breast warmth, with or without a lump that can be felt.
The above-mentioned symptoms have been present for less than 6 months.
The erythema covers at least a third of the breast.
Initial biopsy samples from the affected breast show invasive carcinoma.
Further examination of tissue from the affected breast should include testing to see if the cancer cells have hormone receptors (oestrogen and progesterone receptors) or if they have greater than normal amounts of the HER2 gene and/or the HER2 protein (HER2-positive breast cancer).
Imaging and staging tests include the following:
A diagnostic mammogram and an ultrasound of the breast and regional (nearby) lymph nodes
A PET scan or a CT scan and a bone scan to see if the cancer has spread to other parts of the body
Proper diagnosis and staging of inflammatory breast cancer helps develop the optimal treatment plan and estimate the likely outcome of the disease.
Treatment
Inflammatory breast cancer is generally treated first with systemic chemotherapy to help reduce the size of the tumour. This is followed by surgery to remove the tumour. After the surgery, radiation follows to remove any traces. Is a multimodal approach, which studies have found to be more effective and leads to a possibility of longer survival. Multimodal treatment approach may include:
Neoadjuvant chemotherapy: This type of chemotherapy is given before surgery Usually at least 6 cycles of neoadjuvant chemotherapy is recommend over the course of 4 to 6 months before the tumour is removed surgically. If the disease continues to progress during this time, it is recommended not to delay the surgery.
Targeted therapy: Inflammatory breast cancers often produce greater than normal amounts of the HER2 protein, therefore targeted therapy against this protein may be used to treat the disease. Anti-HER2 therapy can be given both as part of neoadjuvant therapy and after surgery (adjuvant therapy).
Hormone therapy: If the cells of a woman’s inflammatory breast cancer contain hormone receptors, hormone therapy is another treatment option. Drugs which prevent oestrogen from binding to its receptor, and aromatase inhibitors which block the body’s ability to make oestrogen, can cause oestrogen-dependent cancer cells to stop growing and die.
Surgery: The standard surgery for inflammatory breast cancer is a modified radical mastectomy. This involves removal of the entire affected breast and most or all of the lymph nodes under the adjacent arm. Often, the lining over the underlying chest muscles is also removed, but the chest muscles are preserved. Sometimes, however, the smaller chest muscle (pectoralis minor) may be removed.
Radiation therapy: Post-mastectomy radiation therapy to the chest wall under the breast that was removed is a standard part of multimodal therapy. Breast reconstruction can be performed in women with inflammatory breast cancer, but, due to the importance of radiation therapy in treating this disease, experts generally recommend delayed reconstruction.
Adjuvant therapy: Adjuvant systemic therapy may be given after surgery to reduce the chance of cancer recurrence. This therapy may include additional chemotherapy, hormone therapy, targeted therapy or some combination of these treatments.
