[Wellness] Paget's disease awareness

["Karen Patterson" <Patterson@womens-wellness.com>]

Please forward to all of the women in your lives .. Mothers, daughters, 
sisters, aunts, friends, etc.

In November, a rare kind of breast cancer was found. A lady developed a
rash on her breast, similar to that of young mothers who are nursing.

Because her mammogram had been clear, the doctor treated her with 
antibiotics for infections. After 2 rounds, it continued to get worse,
so her doctor sent her for another mammogram. This time it showed a
mass.

A biopsy found a fast growing malignancy. Chemo was started in order to 
shrink the growth; then a mastectomy was performed; then a full round of
Chemo; then radiation. After about 9 months of intense treatment, she
was given a clean bill of health.

She had one year of living each day to its fullest. Then the cancer 
returned to the liver area. She took 4 treatments and decided that she
wanted quality of life, not the after effects of Chemo. She had 5 great
months and she planned each detail of the final days. After a few days 
of needing morphine, she died. She left this message to be delivered to
women everywhere:

Women, PLEASE be alert to anything that is not normal, and be
persistent in getting help as soon as possible.

Paget's Disease: This is a rare form of breast cancer, and is on the
outside of the breast, on the nipple and aureole It appeared as a rash,
which later became a lesion with a crusty outer edge. I would not have 
ever suspected it to be breast cancer but it was. My nipple never seemed
any different to me, but the rash bothered me, so I went to the doctor
for that. Sometimes, it itched and was sore, but other than that it 
didn't bother me. It was just ugly and a nuisance, and could not be
cleared up with all the creams prescribed by my doctor and dermatologist
for the dermatitis on my eyes just prio r to this outbreak. They seemed 
a little concerned but did not warn me it could be cancerous.

Now, I suspect not many women out there know a lesion or rash on the
nipple or aureole can be breast cancer. (Mine started out as a single
red pimple on the aureole. One of the biggest problems with Paget's 
disease of the nipple is that the symptoms appear to be harmless. It is
frequently thought to be a skin inflammation or infection, leading to
unfortunate delays in detection and care.)

What are the symptoms? 

1. A persistent redness, oozing, and crusting of your nipple causing it
to itch and burn (As I stated, mine did not itch or burn much, and had
no oozing I was aware of, but it did have a crust along the outer edge 
on one side.)

2. A sore on your nipple that will not heal. (Mine was on the aureole
area with a whitish thick looking area in center of nipple).

3. Usually only one nipple is effected. How is it diagnosed? Your 
doctor will do a physical exam and should suggest having a mammogram of
both breasts, done immediately. Ev en though the redness, oozing and
crusting closely resemble dermatitis (inflammation of the skin), your
doctor should suspect cancer if the sore is only on one breast. Your
doctor should order a biopsy of your sore to confirm what is going on.

This message should be taken seriously and passed on to as many of your 
relatives and friends as possible; it could save someone's life.

My breast cancer has spread and metastasized to my bones after
receiving mega doses of chemotherapy, 28 treatments of radiation and
taking Tamaxofin. If this had been diagnosed as breast cancer in the 
beginning, perhaps it would not have spread...

TO ALL READERS:

This is sad as women are not aware of Paget's disease. If, by passing
this around on the e-mail, we can make others aware of it and its 
potential danger, we are helping women everywhere.

Please, if you can, take a moment to forward this message to as many
people as possible, especially to your family and friends. It only takes
a moment, yet the results could save a life. 

 

Paget's Disease of the Nipple: Questions and Answers

Key Points

Paget's disease of the nipple is an uncommon type of
cancer that forms in or around the nipple (see Question 1).
Paget's disease of the nipple is almost always associated
with an underlying breast cancer (see Questions 1 and 2).
Scientists do not know exactly what causes Paget's disease
of the nipple, but two major theories have been suggested
for how it develops (see Question 2).
Symptoms of early-stage disease may include redness or
crusting of the nipple skin; symptoms of more advanced
disease often include tingling, itching, increased
sensitivity, burning, or pain in the nipple (see Question
3).
Paget's disease of the nipple is diagnosed by performing a
biopsy (see Question 4).
Surgery is the usual treatment for Paget's disease of the
nipple. Additional treatments may be recommended under
certain circumstances (see Question 5).
Many clinical trials for breast cancer are under way (see
Question 6).




What is Paget's disease of the nipple?

Paget's disease of the nipple, also called Paget's
disease of the breast, is an uncommon type of cancer that
forms in or around the nipple (1, 2, 3). More than 95
percent of people with Paget's disease of the nipple also
have underlying breast cancer; however, Paget's disease of
the nipple accounts for less than 5 percent of all breast
cancers (1). For instance, of the 211,240 new cases of
breast cancer projected to be diagnosed in 2005, fewer than
11,000 will also involve Paget's disease of the nipple
(4).

Most patients diagnosed with Paget's disease of the nipple
are over age 50, but rare cases have been diagnosed in
patients in their 20s (1). The average age at diagnosis is
62 for women and 69 for men. The disease is rare among both
women and men.

Paget's disease of the nipple was named after Sir James
Paget, a scientist who noted an association between changes
in the appearance of the nipple and underlying breast cancer
(1, 5). There are several other unrelated diseases named
after Paget, including Paget's disease of the bone and
Paget's disease of the vulva; this fact sheet discusses
only Paget's disease of the nipple.


What are the possible causes of Paget's disease of the
nipple?

Scientists do not know exactly what causes Paget's disease
of the nipple, but two major theories have been suggested
for how it develops (1, 2). One theory proposes that cancer
cells, called Paget cells, break off from a tumor inside the
breast and move through the milk ducts to the surface of the
nipple, resulting in Paget's disease of the nipple. This
theory is supported by the fact that more than 97 percent of
patients with Paget's disease also have underlying
invasive breast cancer or ductal carcinoma in situ (DCIS)
(1). DCIS, also called intraductal carcinoma, is a condition
in which abnormal cells are present only in the lining of
the milk ducts in the breast, and have not invaded
surrounding tissue or spread to the lymph nodes. DCIS
sometimes becomes invasive breast cancer. Invasive breast
cancer is cancer that has spread outside the duct into the
breast tissue, and possibly intothe lymph nodes under the
arm or into other parts of the body.

The other theory suggests that skin cells of the nipple
spontaneously become Paget cells. This theory is supported
by the rare cases of Paget's disease in which there is no
underlying breast cancer, and the cases in which the
underlying breast cancer is found to be a separate tumor
from the Paget's disease (1).


What are the symptoms of Paget's disease of the nipple?

Symptoms of early Paget's disease of the nipple include
redness and mild scaling and flaking of the nipple skin (1).
Early symptoms may cause only mild irritation and may not be
enough to prompt a visit to the doctor (3). Improvement in
the skin can occur spontaneously, but this should not be
taken as a sign that the disease has disappeared. More
advanced disease may show more serious destruction of the
skin (1). At this stage, the symptoms may include tingling,
itching, increased sensitivity, burning, and pain. There may
also be discharge from the nipple, and the nipple can appear
flattened against the breast (1, 2).

In approximately half of patients with Paget's disease of
the nipple, a lump or mass in the breast can be felt during
physical examination (1). In most cases, Paget's disease
of the nipple is initially confined to the nipple, later
spreading to the areola or other regions of the breast (1,
2). The areola is the circular area of darker skin that
surrounds the nipple. Paget's disease of the nipple can
also be found only on the areola, where it may resemble
eczema, a noncancerous itchy red rash (1). Although rare,
Paget's disease of the nipple can occur in both breasts
(2).


How is Paget's disease of the nipple diagnosed?

If a health care provider suspects Paget's disease of the
nipple, a biopsy of the nipple skin is performed (1, 2, 3).
In a biopsy, the doctor removes a small sample of tissue. A
pathologist examines the tissue under a microscope to see if
Paget cells are present. The pathologist may use a technique
called immunohistochemistry (staining tissues to identify
specific cells) to differentiate Paget cells from other cell
types (1). A sample of nipple discharge may also be examined
under a microscope for the presence of Paget cells (3).

Because most people with Paget's disease of the nipple
also have underlying breast cancer, physical examination and
mammography (x-ray of the breast) are used to make a
complete diagnosis.


How is Paget's disease of the nipple treated?

Surgery is the most common treatment for Paget's disease
of the nipple (1, 2, 5). The specific treatment often
depends on the characteristics of the underlying breast
cancer.

A modified radical mastectomy may be recommended when
invasive cancer or extensive DCIS has been diagnosed (5). In
this operation, the surgeon removes the breast, the lining
over the chest muscles, and some of the lymph nodes under
the arm. In cases where underlying breast cancer is not
invasive, the surgeon may perform a simple mastectomy to
remove only the breast and the lining over the chest muscles
(2, 5).

Alternatively, patients whose disease is confined to the
nipple and the surrounding area may undergo
breast-conserving surgery or lumpectomy followed by
radiation therapy (1, 2, 5). During breast-conserving
surgery, the surgeon removes the nipple, areola, and the
entire portion of the breast believed to contain the cancer.
In most cases, radiation therapy is also used to help
prevent recurrence (return of the cancer).

During surgery, particularly modified radical mastectomy,
the doctor may perform an axillary node dissection to remove
the lymph nodes under the arm (1, 5). The lymph nodes are
then examined to see if the cancer has spread to them. In
some cases, a sentinel lymph node biopsy may be performed to
remove only one or a few lymph nodes. (For more information
about sentinel lymph node biopsies, please see National
Cancer Institute Fact Sheet 7.44, Sentinel Lymph Node
Biopsy: Questions and Answers, at
http://www.cancer.gov/cancertopics/factsheet/Therapy/sentinel-node-biopsy 
on the Internet.)

Adjuvant treatment (treatment that is given in addition to
surgery to prevent the cancer from coming back) may be part
of the treatment plan, depending on the type of cancer and
whether cancer cells have spread to the lymph nodes.
Radiation treatment is a common adjuvant therapy for
Paget's disease of the nipple following breast-conserving
surgery. Adjuvant treatment with anticancer drugs or hormone
therapies may also be recommended, depending on the extent
of the disease and prognostic factors (estimated chance of
recovery from the disease or chance that the disease will
recur).

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