- What is Paget disease of the nipple?
Paget disease of the nipple, also called Paget 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 disease of the nipple also have underlying
breast cancer; however, Paget 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 disease of the nipple (4).
Most patients diagnosed with Paget 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 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 disease of the bone and Paget disease of
the vulva;
this fact sheet discusses only Paget disease of the nipple.
- What are the possible causes of Paget disease of the nipple?
Scientists do not know exactly what causes Paget 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 disease of the nipple. This
theory is supported by the fact that more than 97 percent of patients with
Paget 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 into the 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 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 disease (1).
- What are the symptoms of Paget disease of the nipple?
Symptoms of early Paget 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 disease of the nipple, a lump
or mass in the breast can be felt during physical
examination (1). In most cases, Paget 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 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 disease of the nipple can occur in
both breasts (2).
- How is Paget disease of the nipple diagnosed?
If a health care provider suspects Paget 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 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 disease of the nipple treated?
Surgery is the most common treatment for Paget 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 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).
- Are clinical trials (research studies) available? Where
can people get more information about clinical trials?
Yes. The NCI is currently sponsoring many clinical trials for all types
of breast cancer. These studies are designed to find new treatments and better
ways to use current treatments. As new and improved treatments are found for
breast cancer, the treatment options for Paget disease of the nipple will
also improve (2, 5).
People interested in taking part in a clinical trial should talk with their
doctor. Information about clinical trials is available from the NCI's Cancer
Information Service (CIS) (see below) at 1–800–4–CANCER
and in the NCI booklet Taking Part in Cancer Treatment Research Studies, which can be found at http://www.cancer.gov/publications
on the Internet. This booklet describes how research studies are carried out
and explains their possible benefits and risks. Further information about
clinical trials is available at http://www.cancer.gov/clinicaltrials
on the NCI's Web site. The Web site offers detailed information about specific
ongoing studies by linking to PDQ®,
the NCI's cancer information database. The CIS also provides information from
PDQ.