The facts:
Since I started this blog it occurred to me that I probably
had not informed those of you that wish to know exactly what type of cancer
this is and why it is so lethal to women and some men. There are a huge amount of links out there
that you could go to but below lists the main points. It is important to understand it as it is one
of the rarest and one of the most hard to detect of all breast cancers and so
if this informs and educates then great.
It can be linked to weight something I have battled with my entire life,
but it can also simply be that you are unlucky, a woman, a man, over 50, under
50 etc so this is not the time to over analyse and beat my- self up. It’s just happened so I will deal with it.
So what is inflammatory breast cancer?
Inflammatory Breast Cancer
Key Points
- Inflammatory
breast cancer is a rare and very aggressive disease with symptoms that
include redness, swelling, tenderness, and warmth in the breast.
- Treatment
for inflammatory breast cancer is usually more aggressive than treatment
for most other types of breast cancer.
1.
What is
inflammatory breast cancer?
Inflammatory
breast cancer is a rare and very aggressive disease in which 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 accounts for 1 to 5 percent of all breast cancers diagnosed. 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.
Inflammatory
breast cancer progresses rapidly, often in a matter of weeks or months.
Inflammatory breast cancer is either stage
III or IV at diagnosis, (I have stage III.), depending on whether cancer cells
have spread only to nearby lymph nodes or to other tissues as well.
Additional
features of inflammatory breast cancer include the following:
- Compared
with other types of breast cancer, inflammatory breast cancer tends to be
diagnosed at younger ages
- It
is more common and diagnosed at younger ages in African American women
than in white women.
- Inflammatory
breast tumours are frequently hormone receptor negative, which means that
hormone therapies, such as tamoxifen, that interfere with the growth of
cancer cells fuelled by estrogens may not be effective against these tumours.
- Inflammatory
breast cancer is more common in obese women than in women of normal
weight.
Like other
types of breast cancer, inflammatory breast cancer can occur in men, but
usually at an older age (median age at diagnosis of 66.5 years) than in women.
2.
What are the
symptoms of inflammatory breast cancer?
Symptoms of
inflammatory breast cancer include swelling (edema) 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. In addition, the skin may have ridges or
appear pitted, like the skin of an orange (called peau d'orange).
These symptoms are caused by the build-up of fluid (lymph) in the skin of the
breast. This fluid build-up occurs because cancer cells have blocked lymph
vessels in the skin, preventing the normal flow of lymph through the tissue.
Sometimes, the breast may contain a solid tumour that can be felt during a
physical exam, but, more often, a tumour cannot be felt.
Other
symptoms of inflammatory breast cancer include a rapid increase in breast size;
sensations of heaviness, burning, or tenderness in the breast; or a nipple that
is inverted (facing inward). Swollen lymph nodes may also be present under the
arm, near the collarbone, or in both places.
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. For this reason, women with inflammatory breast cancer
often have a delayed diagnosis of their disease.
3.
How is
inflammatory breast cancer diagnosed?
Inflammatory
breast cancer can be difficult to diagnose. Often, there is no lump that can be
felt during a physical exam or seen in a screening
mammogram. In addition, most women diagnosed with inflammatory breast
cancer have non-fatty (dense) breast tissue, which makes cancer detection in a
screening mammogram more difficult. Also, because inflammatory breast cancer is
so aggressive, it can arise between scheduled screening mammograms and progress
quickly. The symptoms of inflammatory breast cancer may be mistaken for those
of mastitis, which is an infection of the breast, or another form of locally
advanced breast cancer.
To help
prevent delays in diagnosis and in choosing the best course of treatment, an
international panel of experts published guidelines on how doctors can diagnose
and stage inflammatory breast cancer correctly. Their recommendations are
summarized below.
Minimum
criteria for a diagnosis of inflammatory breast cancer include the following:
- A
rapid onset of erythema (redness), edema (swelling), and a peau
d’orange appearance 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 (estrogen and progesterone receptors)
or a mutation that causes them to make greater than normal amounts of the HER2
protein (HER2-positive breast cancer).
Imaging and
staging tests should include the following:
Proper
diagnosis and staging of cancer helps doctors develop the best treatment plan
and estimate the likely outcome of the disease, including the chances for
recurrence and survival.
4.
How is
inflammatory breast cancer treated?
Inflammatory
breast cancer is treated first with systemic
chemotherapy to help shrink the tumor, then with surgery to remove the tumour,
followed by radiation
therapy. This approach to treatment is called a multimodal approach.
Studies have found that women with inflammatory breast cancer who are treated
with a multi-modal approach have better responses to therapy and longer
survival. Treatments used in a multimodal approach may include those described
below.
- Neoadjuvant
chemotherapy: This type of chemotherapy is given before surgery and
usually includes both anthracycline
and taxane
drugs. At least six cycles of neoadjuvant chemotherapy given over the
course of 4 to 6 months before attempting to remove the tumour has been
recommended, unless the disease continues to progress during this time
and doctors decide that surgery should not be delayed.
- Targeted
therapy: This type of treatment may be used if a woman’s biopsy samples
show that her cancer cells have a tumour marker that can be targeted with
specific drugs. For example, inflammatory breast cancers often produce
greater than normal amounts of the HER2 protein, which means they may
respond positively to drugs, such as trastuzumab
(Herceptin), that target this protein. Anti-HER2 therapy can be given as
part of neoadjuvant therapy and after surgery (adjuvant therapy). Studies
have shown that women with inflammatory breast cancer who received
trastuzumab in addition to chemotherapy have better responses to
treatment and better survival.
- Hormone
therapy: If a woman’s biopsy samples show that her cancer cells contain
hormone receptors, hormone therapy is another treatment option. For
example, breast cancer cells that have estrogen receptors depend on the
female hormone estrogen to promote their growth. Drugs such as tamoxifen,
which prevent estrogen from binding to its receptor, and aromatase
inhibitors such as letrozole,
which block the body’s ability to make estrogen, can cause
estrogen-dependent cancer cells to stop growing and die.
- Surgery:
The standard surgery for inflammatory breast cancer is a modified radical
mastectomy. This surgery 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, too.
- Radiation
therapy: Post-mastectomy radiation therapy to the chest wall under the
breast that was removed is a standard part of multi-modal therapy for
inflammatory breast cancer. If a woman received trastuzumab before
surgery, she may continue to receive it during postoperative radiation
therapy. If breast
reconstruction is planned, the sequencing of the radiation therapy
and reconstructive surgery may be influenced by the method of breast
reconstruction used. If a breast implant is to be used, the preferred
approach is to delay radiation therapy until after the reconstructive
surgery. If a woman’s own tissues are going to be used in breast
reconstruction, it is preferable to delay reconstructive surgery until
after the radiation therapy has been completed.
- Adjuvant
therapy: Adjuvant systemic therapy may be given after surgery to reduce
the chance of cancer recurrence. This therapy may include additional
chemotherapy, antihormonal therapy, targeted therapy (such as
trastuzumab), or some combination of these treatments.
- Supportive/palliative
care: The goal of supportive/palliative care is to improve the quality of
life of patients who have a serious or life-threatening disease, such as
cancer, and to provide support to their loved ones.
5.
What is the
prognosis of patients with inflammatory breast cancer?
The
prognosis, or likely outcome, for a patient diagnosed with cancer is often
viewed as the chance that the cancer will be treated successfully and that the
patient will recover completely. Many factors can influence a cancer patient’s
prognosis, including the type and location of the cancer, the stage
of the disease, the patient’s age and overall general health, and the extent to
which the patient’s disease responds to treatment.
Because
inflammatory breast cancer usually develops quickly and spreads aggressively to
other parts of the body, women diagnosed with this disease, in general, do not
survive as long as women diagnosed with other types of breast cancer. According
to statistics from NCI’s Surveillance, Epidemiology, and End Results (SEER)
program, the 5-year relative survival for women diagnosed with inflammatory
breast cancer during the period from 1988 through 2001 was 34 percent, compared
with a 5-year relative survival of up to 87 percent among women diagnosed with
other stages of invasive breast cancers.
It is
important to keep in mind, however, that these survival statistics are based on
large numbers of patients and that an individual woman’s prognosis could be
better or worse, depending on her tumor characteristics and medical history.
Women who have inflammatory breast cancer are encouraged to talk with their
doctor about their prognosis, given their particular situation.
Research has
shown that the following factors are associated with a better prognosis for
women with inflammatory breast cancer:
- Stage
of disease: Women with stage III disease have a better prognosis than
women with stage IV disease. Among women who have stage III inflammatory
breast cancer, about 40 percent survive at least 5 years after their
diagnosis, whereas among women with stage IV inflammatory breast cancer,
only about 11 percent survive for at least 5 years after their diagnosis.
- Tumour
grade: Women with grade I or grade II tumours have a better prognosis
than those with grade III tumours. Tumour grade is a term that describes
what cancer cells look like under a microscope, with a higher grade
indicating a more abnormal appearance and a more aggressive cancer that
is likely to grow and spread. Among women who are diagnosed with grade I
or grade II inflammatory breast cancer, 77 percent survived at least 2
years after their diagnosis, whereas among women who were diagnosed with
grade III inflammatory breast cancer, 65 percent survived at least 2
years after their diagnosis.
- Ethnicity:
African American women who have inflammatory breast cancer generally have
a worse prognosis than women of other racial and ethnic groups. Studies
have found that around 53 percent of African American women who are
diagnosed with inflammatory breast cancer survive at least 2 years after
diagnosis, whereas 69 percent of women from other racial and ethnic
groups survive at least 2 years after diagnosis.
- Estrogen
receptor status: Women with inflammatory breast whose cancer cells have
estrogen receptors have a better prognosis than those whose cancer cells
are estrogen receptor negative. The median survival for women with
estrogen-receptor negative inflammatory breast cancer is 2 years, whereas
the median survival for those with estrogen receptor-positive
inflammatory breast cancer is 4 years.
- Type
of treatment: Multimodal treatment of inflammatory breast cancer improves
a woman’s prognosis. Historically, among women who had only surgery,
radiation therapy, or surgery and radiation therapy, fewer than 5 percent
survived longer than 5 years. However, when women are treated with
neoadjuvant chemotherapy, mastectomy, adjuvant chemotherapy, and
radiation therapy, their 5-year disease-free survival ranges from 24 to
49 percent. One long-term study found that 28 percent of women with
inflammatory breast cancer survived 15 years or longer after they were
treated with multimodal therapy.
Ongoing research,
especially at the molecular level, will increase our understanding of how
inflammatory breast cancer begins and progresses. This knowledge should enable
the development of new treatments and more accurate prognoses for women
diagnosed with this disease. It is important, therefore, that women who are
diagnosed with inflammatory breast cancer talk with their doctor about the
option of participating in a clinical trial.
