Inflammatory breast cancer (IBC) is a rare and very aggressivetype of breast cancer that tends to spread quickly, even in the relatively early-stages.
Interestingly, the signs and symptoms of IBC are quite common. However, these signs and symptoms are most often associated with benign breast conditions.
It is necessary to take the symptoms of inflammatory breast cancer very seriously, even if your doctor suspects a benign condition.
Breast cancer testing should always be initiated for women with symptoms of IBC, particularly young women and pregnant or breastfeeding ones.
Inflammatory breast cancer is one of the most aggressive types of breast cancer and prognosis is generally poorer than for other ‘advanced stage‘ breast cancers.
Life is a journey that must be travelled no matter how bad the road or the accommodations.
Lovely quote, Moose!
Incidence Rates of Inflammatory Breast Cancer
The rarity of inflammatory breast cancer (IBC) means that large progressive research studies are few and far between.
Hence, the level of progress and knowledge for IBC does not match that of other types of breast cancer.
The incidence rate of IBC varies from country to country and amongst different ethical groups. However, the general incidence rate of IBC is only around 1% to 5% of all newly diagnosed breast cancers in American women.
According to one medical study, the incidence of IBC is higher in North African countries, particularly Algeria, Egypt, Tunisia and Morocco.
In the above mentioned African countries, incidence rates are between 10% and 15%. In comparison, the lowest incidence rates of IBC are in North America.
Inflammatory Breast Cancer, Ethnic Groups and new Research
Similarly to other breast cancers, the incidence rate of inflammatory breast cancer varies according to ethnic groups in the United States.
The rate of IBC is around 1.3 per 100,000 across all groups of women. However, African American women have a higher risk of 1.6 per 100,000. Asian and Pacific Islanders have the lowest risk of IBC at 0.7 per 100,000. Scientists still do not fully understand the differences in risk for breast cancer between different countries.
Furthermore, a diagnosis of IBC tends to be at a younger age than other breast cancers. The average age at diagnosis of IBC is the mid to late ’50s. Most women are post-menopausal at diagnosis. Interestingly, a high Body Mass Index (BMI) is thought to significantly increase the odds of developing IBC.
The latest research is investigating the mammary tumor virus as a potential risk factor for Inflammatory Breast Cancer.
What is the mammary tumor virus?
Mouse mammary Tumor Virus (MMTV) is a milk-transmitted retrovirus that is thought to cause infection and affect the immune response.
Mortality Rates and the Good News
In the past, IBC has a poor survival rate. Indeed, the 5-year overall survival rate was less than 5% with a median rate of just 15 months. One of the reasons for the low survival rate is that IBC is often at a late stage (III or IV) at the diagnosis.
Sadly, IBC has often already spread to the lymph nodes on diagnosis.
However, according to a more recent research study, over the last 30 years survival rates for IBC have improved significantly. The 15 year survival rate is now around 20% to 30%.
Specialists believe that the improvement in survival rates for breast cancer is due to changes in treatment.
These changes include:-
A multidisciplinary approach
Preoperative chemotherapy surgery
Radiation treatment.
An improvement in the understanding of IBC on a molecular level over the last ten years.
In addition, a 2015 study compares survival trends of women with inflammatory breast cancer (without metastatic spread, so early-stage) before and after the year 2006. The 3-year survival rate for those treated for IBC before October 2006 was around 63%. In comparison, for cases of IBC after 2006 the 3-year survival rate has risen to 82%.
The above statistics, are again, a testimony to the improvement in targeted treatment, in this case, particularly HER-2 therapy.
That is really good news for women with Inflammatory Breast Cancer.
Yes, but please remember all survival statistics are generalizations only and every case is individual.
Clinical Symptoms of Inflammatory Breast Cancer
The symptoms of inflammatory breast cancer are not typical symptoms of breast cancer. Indeed, the symptoms are very similar to breast mastitis or a breast infection.
The Symptoms include:-
Skin Changes: Patches of skin on the breast appear red or pink in color, or appear bruised
The texture of the affected area of the breast may also change and appear dimpled or pitted, a little like the skin of an orange
Itchy breasts: Itchiness around the affected area can occur
Swelling of the breast: You may notice that one breast is larger than the other
Breast pain: The affected breast may be tender, painful or feel heavier
The breast feels hot to the touch or there can be a burning sensation
Ridges or welt like marks may appear on the skin
Inverted nipple, dimpling or flattening of the nipple
Nipple Discharge
Swollen lymph nodes under the arm or in the neck
Women with inflammatory breast cancer will typically present to a physician with a rapidly progressing, firm, tender, and enlarged breast. If you have even a small patch of inflamed, red skin on breast self-exam it is important to see your physician straight away.
Notably, women with inflammatory breast cancer will tend not to have a fever. Because it is a cancer that spreads very rapidly into and around the breast tissue, women with IBC might present with axillary adenopathy (enlarged axillary lymph nodes). This can be a sign that cancer has spread (metastatic breast cancer).
Oh no Doc, that photo looks painful.
Yes, but once again, any unusual symptoms of the breast should be checked out straight away.
Diagnosis of inflammatory breast cancer can be inconclusive at first
The cause of the appearance of the breast in IBC is the invasion of the skin lymphatics by breast cancer cells. The obstructed lymph vessels are responsible for producing the characteristic skin changes that can mimic a benign inflammatory process.
Standard diagnostic tests for breast cancer screening include:-
However, diagnosis can be very difficult for IBC and even the above diagnostic tests do not always diagnose IBC.
Diagnosis is hard and that goes both ways.
Huh?
Screening tests for IBC can say there is no cancer when, in fact, there is. The tests can be wrong at first, and sometimes this leads to tragedy.
On the other hand, I’ve also seen cases where biopsy has called it inflammatory cancer, but it all just went away.
Inflammatory Breast Cancer: The Diagnosis
So, frequently in the diagnosis of IBC a PET scan is utilized.
Furthermore, larger sample excisional biopsies are often necessary. A skin biopsy can find evidence of invasive breast cancer cells, but this is not always the case.
Inflammatory breast cancer has a tendency to grow in ‘layers‘, so it may be quite a while before a palpable lump actually appears. Furthermore, if a lump develops it might appear quite suddenly.
Around 30% of inflammatory breast cancers never develop an actual breast lump.
Recent studies have pointed to the potential advantages of new diagnostic techniques, such as fluorodeoxyglucose positron emission tomography (FDG PET).
What is an FDG-Pet? Am I one, Doc?
A FDG-PET is an imaging technique that uses a radioactive drug that shows the differences between diseased tissue and healthy cell tissues. The scan produces a 3-D image.
Inflammatory Breast Cancer and Hormone Receptor Status
Inflammatory breast cancer tumors often have increasedangiogenesis and lymphangiogenesis.
Furthermore, IBC tumors also frequently show an overexpression of HER-2, RhoC GTPase, and NF-{kappa}B genes.
Inflammatory breast cancers are more likely to be negative for Estrogen receptor status (ER-) and/or progesterone receptor status (PR-).
Inflammatory breast cancer tumors have a higher frequency of ER- and PR- tumors in comparison to other advanced breast cancer tumors. Indeed, some studies show that up to 83% of IBC tumors are ER-. This tends to affect the efficacy of treatment as the tumors do not respond to hormone therapy.
Data from the Surveillance, Epidemiology and End Results (SEER) database show a higher median survival rate in inflammatory breast cancer ER+ tumors (4 years) compared with ER- tumors (2 years).
… and there’s me thinking that ER is the emergency room. As for PR … I won’t even go there.
Mammographic and ultrasound features of inflammatory breast cancer
The typical findings on screening for inflammatory breast cancers are thickening of the skin and connective tissues and an increase in breast density.
In around 30% of inflammatory breast cancers casesthere is no lump (or tumor) . Rather IBC usually presents as a ‘diffuse infiltration‘ of cancer cells, so it is not as easily detected on mammogram or ultrasound.
So, the absence of a true breast mass on mammography does not always rule out cancer.
In addition, the high density of the breast might hide an actual tumor deeper within the breast.
Ultrasound can be helpful in the diagnostic process, as it may be able to detect masses hidden at mammography and on clinical examination.
Ultrasound is also useful to detect axillary adenopathy (swelling under the armpits) and this can help with taking more accurate biopsy samples. With inflammatory breast cancer, ultrasound images might show edema and skin thickening along with an ill-defined mass of some kind.
Well, at least there is some good news.
Ultrasound is so much more comfortable than a mammogram.
Yes, if I ever find the guy who invented the mammogram I know exactly how to screen him for testicular cancer.
Management and treatment options for inflammatory cancer of the breast
For some women with IBC, the initial investigations to find a diagnosis may not confirm a benign or a malignant condition.
So, a patient may be given conservative treatments such as anti-inflammatory drugs and antibiotics. Monitoring of the response to antibiotic therapy is very important if symptoms do not improve further investigations will be necessary.
Sometimes, a large excisional breast biopsy is needed to really figure out what is going on.
Inflammatory breast cancer cells tend to grow widely through the tissues of the breast, rather than as a single tumour. For this reason, chemotherapy drugs or radiation therapy are often given before surgery.
Apparently, quite a few women with IBC experience pain in the breast.
Yes, that is true. However, one study suggests that pain and tenderness are experienced in up to 49.3% of women with invasive breast cancer.
Treatment should begin without delay
Inflammatory breast cancer needs treatment as soon as possible. However, because of the unique properties it is recommended that an oncologist with experience in inflammatory breast cancer heads the treatment and management team.
Management of inflammatory breast cancer requires a combined-modality approach to therapy.
Remember ladies, you beat cancer by how you live, why you live and in the manner you live.
Risk of recurrence and metastatic breast cancer
Due to the involvement of the skin in IBC, the risk for local recurrence probability of lymph node metastasis is very high.
For this reason, breast physicians rarely suggest an immediate mastectomy and instead will prescribe a course of preoperative chemotherapy.
Also, your physician will usually perform a full staging workup. A work-up involves chest x-ray scans, bone scans, and even abdominal ultrasound scans.
After chemotherapy, some patients may then undergo a full or modified radical mastectomy, along with axillary lymph node dissection.
However, if the medical treatment with chemotherapy gets a dramatically good response, it may not be necessary to remove the breast with surgery.
After surgery, treatment with radiation therapy to the regional lymph nodes and to the chest wall usually follows.
Finally, women will likely receive endocrine therapy or other targeted therapies specific to the hormone receptor status.
The active stage of treatment for inflammatory cancer of the breast is intensive and difficult and usually takes about 1 year to complete.
Future Research on Inflammatory Breast Cancer
Recent research on inflammatory breast cancer has focused on the work on genetic determinants that underlie the inflammatory breast cancer phenotype.
Indeed, scientists have already identified some of the genes that are present in the development and progression of this disease. However, there has been no real ‘genetic‘ breakthrough in the treatment of the disease.
For the most part, inflammatory breast cancer has a distinct molecular profile, though it does share some genetic changes with other breast cancers.
Inflammatory breast cancer is a truly awful condition.
As an IBC patient you can find out clinical trials to participate that may also help others in the future.
Quick Question and Answer
How quickly does inflammatory breast cancer spread, develop and grow?
The skin pinkness (rash) spreads very quickly sometimes over a matter of several months. As mentioned earlier, IBC is an aggressive type of breast cancer that spreads very quickly. Increased awareness of the symptoms and early diagnosis is key.
Where does inflammatory breast cancer spread to?
Like other breast cancers IBC spreads to the axilla and internal mammary lymph nodes. There is also often lymphovascular invasion. Often it can spread into the bone.
Why is inflammatory breast cancer so deadly?
Because of the tendency for misdiagnosis. IBC is often mistaken for mastitis, or sometimes even shingles. On other occasions, IBC may be mistaken for a different type of locally advanced breast cancer and not given the right treatment.
The difficulty of diagnosis in combination with the fast growth of cancer often means IBC is at an advanced stage at diagnosis.
What causes inflammatory breast cancer?
The causes of inflammatory breast cancer are still not fully understood. However, viral infection, genetic factors and obesity are all thought to have a link.
I have inflammatory breast cancer and have been told that I need neoadjuvant chemotherapy, what does this mean?
Neoadjuvant therapy actually means any medical therapy that is given before surgery to shrink the tumor or halt or slow down the disease process.
Neoadjuvant therapy is usually drug treatments such as hormone therapy (for example, Tamoxifen), targeted therapy, chemotherapy or radiation therapy.
Which other sites have good information on inflammatory breast cancer?
Some of these sites will also be able to advise you on up-to-date clinical trials if you are newly diagnosed with breast cancer.
What happens after you finish initial treatment for Inflammatory Breast Cancer?
Well, you can relax and congratulate yourself that you have come this far. Ongoing monitoring and screening will be necessary to detect any recurrence or further spread. It is important to talk to your oncologist about regular check-ups and tests necessary after the initial treatment.
Furthermore, on top of conventional medicines, it may be helpful to find a group for emotional support. Many women find complementary therapies to help ease symptoms and improve quality of life.
Herbal medicines: Although please check the safety of any drugs that you take. In addition, ensure that herbal medicines do not react with your treatment, such as chemotherapy
Massage
The most important thing to remember in Inflammatory Breast Cancer is that early detection is SO important. All skin changes even new freckles should be checked out.
Yes, and don’t forget the best treatments are sunshine, air, exercise, water, diet, rest and laughter,
Dawood S, Massimo Cristofanilli M. (2011) Inflammatory Breast Cancer: What Progress Have We Made? Review Article | March 15, 2011 | Oncology Journal, Breast Cancer https://www.ncbi.nlm.nih.gov/pubmed/21548470
Tsai CJ, Li J, Gonzalez-Angulo AM, Allen PK, Woodward WA, Ueno NT, Lucci A, Krishnamurthy S, Gong Y. (et al). (2015) Outcomes After Multidisciplinary Treatment of Inflammatory Breast Cancer in the Era of Neoadjuvant HER2-directed Therapy Am J Clin Oncol. 2015 Jun;38(3):242-7. https://www.ncbi.nlm.nih.gov/pubmed/23648437.
Dr Halls has 25 years experience as a radiologist. He worked for 13 years at Cross Cancer Institute in Edmonton, a world-class cancer treatment facility. He has had high-volume experience with cancer, interventional procedures, clinical trials and his own phase 1 and 2 research in MRI and breast cancer staging.