Disease Of The Nipple
Rare Expression of Breast Cancer
Paget's disease typically
appears as a red, scaly
rash around the nipple.
Under the microscope,
cells form in clusters
near the surface.
years ago, there was an e-mail circulating on the Internet that began:
November I lost my sister (Betty Botts of Troy, Al.) to a rare kind
of cancer. She developed a rash on her breast similar to that of young
mothers who are nursing."
on to send a harsh warning about Paget's Disease.
No one was able to verify if any of the women named in the e-mail exist.
However, Paget's Disease is real. It is an extremely rare, and not normally
deadly, form of breast cancer. It does need to be diagnosed and treated.
of the disease that the e-mail describes sounds much closer to the progression
of Inflammatory Breast
Cancer (IBC). IBC is also rare, but it is a very aggressive form
of breast cancer, which also begins as what looks like a rash on the
breast. IBC is always diagnosed at Stage III or Stage IV and needs to
be treated quickly with the appropriate therapies to delay or prevent
the spread to other organs.
Is Paget's Disease of the Nipple?
The real name of the disease is "Paget's Disease of the Nipple,"
"Paget's Disease of the Breast" or "Mammary Paget's Disease."
It is named
after the English surgeon, Sir James Paget, who first described it in
the 1800s. Sir James Paget has a bone disease named after him - also
called Paget's Disease. There is no connection between these diseases,
except for the fact that they were both first described by Sir James
disease occurs in 14% of female patients with breast cancer and
may be the only indication of underlying breast cancer. The cancer that
it is normally associated with is Ductal Carcinoma In Situ (DCIS).
get Paget's disease but this is extremely rare. It may be associated
with Klinefelter's Syndrome, a genetic abnormality that can cause enlarged
breasts in men (gynecomastia).
of the symptoms are malignant Paget's cells in the ductal tissue underneath
the nipple. The skin (epidermis) becomes involved.
half of the cases of Paget's disease there is a lump which indicates
that the underlying cancer is more developed.
is no lump, the cells are probably limited to the ducts and the skin
and the chances of other organs being involved is very small.
of Paget's Disease of the Nipple
It is called Paget's Disease of the nipple because that it where
the symptoms first appear.
common signs are:
- A red,
scaly rash on the nipple
rash may be itchy or you may notice a burning feeling
- It may
spread to the darker area around the nipple (areola)
rash may become sores that are weepy - that leak fluid
- It may
become crusty due to the discharge
nipple may become inverted
may be discharge from the nipple
may be diagnosed and treated as a skin rash. If you suspect Paget's
look for signs that it is not a common rash.
rashes will spread to both breasts - Paget's does not.
skin rashes will affect the areola and outer skin, Paget's begins
at the nipple.
Paget's Disease of the Nipple
The disease is difficult to diagnose. Paget's may be a precancerous
condition or it may involve cancer. If it is precancerous, it does not
show on many standard tests.
tests for diagnosing Paget's are examining the nipple discharge, mammograms
and sonograms and MRIs. The results on the mammograms and sonograms
and MRIs will usually come back negative if the underlying cancer is
not well developed. This will happen about 50 percent of the time.
many cases, the discharge from the nipple can be examined to see
if there are any Paget's cells present.
only sign of Paget's may be a thickening in the area of the nipple
and areola. If the cancer is detected, it can be anywhere in the
breast. It does not necessarily have to be directly beneath the
your health care provider suspects Paget's, you will probably
need to have a biopsy taken to confirm the diagnosis. This involves
taking a small section of skin and breast tissue from the affected
area. This is usually done in the doctor's office with a local
anesthetic. The tissue is then examined by a pathologist.
states: "The neoplastic [cells that have begun to exhibit abnormal
behavior] are most numerous in the basal [lower] portion of the nipple
epidermis [skin]; therefore, a full thickness biopsy of the skin, rather
than a shave biopsy, should be used for diagnosis."
For Paget's Disease of the Breast
Treatments depend on whether there is a diagnosis of cancer. Since
approximately 50 percent of the cases of Paget's found are precancerous,
they will require less treatment and have a much better prognosis.
you will need surgery and what type of surgery your doctor recommends
will depend on the underlying cells. If there is a cancer diagnosis,
the treatment will be based on the cancer diagnosis - not the
Paget's. An early stage cancer will probably involve a lumpectomy,
removing the lump and the breast tissue around it. A more advanced
cancer may require a mastectomy, removal of the breast, and other
you dont have a lump, a wide local excision, removing the
tissue that is involved, followed by radiotherapy may be suggested.
invasive cancer it is important to find out whether the cancer
has spread to the lymph nodes in the axilla (glands in your armpit).
This may mean removal of the lymph nodes or a Sentinel Node Biopsy.
you have any questions on these procedures, you can access articles
by typing the information into the search box. This will find
all of the information available on this site, the rest of the
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many cases, your health care team will suggest that you have radiotherapy
to make sure that any local cancer cells that may have escaped
the surgeon's scalpel are killed. If there was no underlying cancer
detected, this will probably be the only treatment you require
after surgery. In some cases, Paget's is treated with radiation
alone and no surgery is necessary. If you opt for a mastectomy,
you may not need radiation treatments.
- These adjuvant
treatments may be prescribed by your health care team to treat
the cancer that produced the Paget's symptoms. There are many
different drug therapies available. These treatments may involve
drugs such as tamoxifen, which are meant to prevent the cancer
from growing again. You may need more aggressive treatments if
the cancer has spread to your lymph nodes or other sites.
The prognosis with Paget's Disease depends on the diagnosis. If Paget's
is diagnosed and treated while it is precancerous the survival rate
is extremely high. While the cancer underlying the symptoms is in an
early stage, the survival rate is around 95 percent, still very high.
The more advanced and widespread the cancer is, the less favorable the
suspect that you have the condition, speak to your doctor. Remember
that it is extremely rare. This works both for and against you. As with
any rare cancer, you may have discovered more about the disease through
your research than your doctor is aware of. If you feel that you need
a mammogram or a biopsy, do not be shy - request the treatments from
your health care provider. However, don't be upset if your doctor disagrees
doctor has had any experience with this disease, you will probably get
the full work up - unless there are strong indications that you really
do just have a rash or an infection of some sort.
doctor is not familiar with the condition, try to get a second opinion
from someone who has treated Paget's.
mind that Paget's normally indicates either a precancerous condition
or a nonaggressive type of cancer that is in the earliest stages.
Last updated: October 23, 2006
Also see -> Paget's
Disease of the Nipple Research Studies
on the Web:
disease of the breast
Disease of the Nipple: Questions and Answers
Disease of the Nipple (Breast Cancer)