Paget’s disease of the breast

Paget’s disease is a specific kind of neoplastic (new cell) growth which begins with the nipple and gradually spreads to other areas of the areola and beyond.

This is a serious condition as it is almost always associated with ductal carcinoma (breast cancer)

The condition was originally reported by Sir James Paget in 1874. Paget was an English surgeon, and he also discovered a bone-related condition, ‘Paget’s disease of the bone’, but these two conditions which bear his name are completely medically unrelated.

Please note. This page is a little old, but still fantastic. However, we do have a new section about Paget’s disease here. Check it out if you fancy.

Paget’s disease can be mistaken for a mild skin irritation

Initially, this condition might resemble a benign skin condition, such as:-

eczema
contact dermatitis
other benign breast neoplasms
duct ectasia
nipple adenoma.

So, there is some danger that physicians may initially dismiss the presenting symptoms but finally discover the disease at a more advanced stage.

Specialists consider this disease to be a form of breast cancer. Furthermore, this type of breast cancer accounts for between 3% to 4% of all breast cancers.

Paget’s disease typically results from an extension of an underlying ductal carcinoma in situ (DCIS) or invasive carcinoma into the nipple and areola region.

The Symptoms

Symptoms can include several inflammatory skin and nipple changes, but the most common symptom is bloody nipple discharge.

Other symptoms may include:-

Pain and itching
a type of wound that won’t heal (ulceration)
scaling of the skin
erythema (red rash)
nipple retraction – more common as the disease progresses.

But in about 10% of cases, there are no clinical symptoms at all in the early stages. In many instances, the nipple itself may appear completely normal, despite the presence of other clinical features.

Just under 50% of women will present with a palpable breast lump. However, of those women without any obvious breast lump, about 50% will not show a mass on a mammogram.

Do not worry though because so many different conditions can cause a bloody discharge.

Why can Paget’s disease be difficult to diagnose?

So, sometimes, there is NO:-

palpable lump
mass on a mammogram
obvious or destructive nipple changes

So, it is easy to see how breast cancer specialists can often too easily dismiss, or misdiagnose this disease, especially if it occurs in both breasts (bilaterally).

All patients with uncertain clinical symptoms of Paget’s disease should undergo mammography and then a biopsy to confirm or exclude the diagnosis.

Histological Evaluation and diagnosis

So, Paget’s disease is essentially an infiltration of the nipple-areola complex epidermis (outer skin layers) by adenocarcinoma cells (derived from glandular epithelium).

Thus the cytological analysis should reveal malignant cell infiltration in the dermis. These malignant cells (Paget’s Cells) are typically large and with high-grade pleomorphic nuclei.

The abnormal cells are often arranged in single units or confluent nests within the epidermis. (They tend to cluster in lower layers and occur singly in the outer, superficial layers).

Paget’s cells will often stain a pale color, and the surrounding cytoplasm is typically clear and abundant.

Mammographic evaluation of Paget’s breast disease

As mentioned, about 50% of all cases of Paget’s disease do not reveal a mass on mammographic X-ray. There are no unique mammographic features corresponding to a Paget’s mass.

So, if medics discover a tumor it would be characterized according to the type of ductal carcinoma it resembles.

However, the mammographic evaluation can help show the extent of whole breast involvement, (i.e. invasive breast cancer) and can guide and influence treatment.

Once there is a definite diagnosis of Paget’s disease specialists tend to treat it in a highly aggressive fashion. Magnetic resonance imaging (MRI) tends not to be useful for Paget’s disease evaluation due to its non-specificity. MRI is useful in detecting occult (hidden) breast neoplasia, but not all of Paget’s disease cases will feature cell neoplasia.

Really, the diagnosis of Paget’s disease has to be by looking directly at the cells. An experienced radiologist, however, might be able to detect small changes to the inner structure of the nipple-areolar complex, which may be suspicious of Paget’s disease and make the case for a full histological evaluation.

Treatment of Paget’s disease of the Breast

Breast cancer specialists consider Paget’s disease to be a serious and aggressive form of breast cancer and the first line of treatment is usually surgery.

Up until recently, surgeons tend to proceed with a full mastectomy and the typical range of axillary (shoulder-region lymph nodes) checks, regardless of whether or not there was a palpable lump.

However, when a lump is present it really is a form of invasive breast cancer, but without a palpable lump, the surgical interventions may be a little more conservative.

Paget’s disease without a palpable lump is really a kind of ductal carcinoma in situ, (DCIS). There is a greater understanding now about DCIS in terms of histological and radiological predictors of cancer aggressiveness, and full mastectomy may not be necessary.

However, this has to be balanced against the unfortunate tendency for Paget’s disease to have a high rate of local recurrence.

Treatment of Paget’s disease is by conservative surgical excision

For patients with a palpable lump, a more conservative surgical excision approach is sometimes adopted. It is very important for surgeons to achieve wide, clear margins.

Surgical excision is usually followed up by tissue sampling from adjacent quadrants and axillary node dissection. Treatment for some patients, who have no lump and a normal mammogram, is by excision of the nipple-areola complex alone.

This approach is typically combined with postoperative radiotherapy, and with good results (low recurrence rates). Additional treatment by radiotherapy or chemotherapy will typically be based on a patient’s age and nodal status.

Paget’s disease tends to recur as invasive breast cancer

Treatment of Paget’s disease can be a tough call, depending to a certain extent, on the risks that both the patient and surgeon are willing to take to preserve the breast.

The concern is, that when Paget’s disease does recur locally, it tends to recur in an invasive context, (spreading into the breast ducts) and generally speaking, the prognosis is better if you can treat breast cancer before it reaches that stage.

The prognosis for women with Paget’s breast disease

The prognosis for Paget’s disease tends to depend upon the status of the underlying breast disease (breast cancer). Generally speaking, the prognosis is much better for women without a palpable lump.

The 10-year survival rate is estimated at between 80% to 90%. Positive axillary node metastases is relatively rare, at less than 13%.

The prognosis and survival rate for Paget’s disease with a palpable mass are generally about the same as for generic infiltrating ductal carcinoma with a five-year survival rate of about 40% and a 10-year survival rate of about 20%.

However, there is no difference in the survival rate for patients with invasive breast cancer with or without Paget’s disease.

A reminder that this page is a little bit old and this last paragraph about treatment and survival is out-of-date. Treatment results are really improved since this page was written.

Here are a bunch of quick Q&A’s for you to go over…

At what age does Paget’s disease of the breast occur?

It most often occurs in women ages 50 and older.

What are the known signs and symptoms of Paget’s disease?

Paget’s disease of the breast affects your nipple and usually the areola surrounding it. It’s simple to mistake the signs and symptoms of this type of disease for skin irritation (dermatitis) or another noncancerous (benign) skin conditions.

Possible signs and symptoms may include:-

flaky or scaly skin on the nipple
crusty, oozing or hardened skin resembling eczema on the nipple, areola or both
itching
redness
tingling or burning sensation
straw-colored or bloody nipple discharge
a flattened or inverted nipple
lump in the breast
thickening skin on the breast
Signs and symptoms usually occur in one breast only

When should I see a doctor?

Check your nipple and areola on both breasts on a regular basis, such as during breast self-exams. If you feel a lump or if you experience itching or skin irritation that persists for more than a month, see your doctor.

If you’re being treated for a skin injury on your breast, and the condition doesn’t go away with treatment, make a follow-up appointment with your doctor as soon as possible. You may need a biopsy to evaluate the affected area.

What causes Paget’s disease?

Doctor’s do not know what causes Paget’s disease of the breast. The most widely accepted theory is that the disease results from underlying ductal breast cancer.

The cancer cells from the original tumor then travel through the milk ducts to the nipple and the surrounding skin. Another theory is that the disease can develop independently in the nipple.

What are the risk factors associated with Paget’s disease?

Risk factors that affect your likelihood of developing Paget’s disease of the breast are the same factors that affect your risk of developing any other type of breast cancer.

These include:-

age
a personal history of breast cancer
history of breast abnormalities
your family history
genetic predisposition
dense breast tissue
radiation exposure
excess weight
hormone replacement therapy (HRT)
race

What can I do to prepare for my doctor’s appointment?

A few things you can do to prepare for your appointment would be to:-

write down any symptoms you may be experiencing
note the key personal information
make a list of all your medications that you are presently taking
consider taking a family member or a friend along with you for your own comfort and support
write down any questions you have in mind that you are wanting to ask your doctor

How is Paget’s disease diagnosed?

Clinical breast exam and physical exams
mammography
breast biopsy
sentinel lymph node biopsy

What kind of treatment and drugs that are recommended to be taken when you have Paget’s disease?

You will likely need surgery. The type of surgery depends on the condition of the skin around your nipple and how advanced the underlying cancer is. Surgical options include:-

simple mastectomy
lumpectomy
adjuvant therapy (such as additional treatment, chemotherapy, radiation therapy, or hormone therapy)

How can you prevent Paget’s disease?

There are a few things you can do to help prevent your chances of developing breast cancer. Some things, however, are beyond your control. These include:-

age
genetics
family history

However, there are some common sense things that you can do to reduce your risk of breast cancer:-

getting enough exercise
limiting alcohol
eating healthy food and maintaining a healthy diet
maintain a healthy weight
some interventions may help reduce your risk as well. For example, preventive medications and risk-reducing surgery.

References

Jamali F R, Ricci A, Deckers P J. Paget’s disease of the nipple-areola complex. Surg Clin North Am 1996: 76: 365-381.
Menendez, M., Menendez, C., Anzalone, G., Paget’s Disease of the Breast Mimicking a Chronic Wound. Wounds. 2008;20(12)
Paone JF, Baker RR. Pathogenesis and treatment of Paget’s disease of the breast. Cancer. 1981;48(3):825-829.
Marshall JK, Griffith KA, Haffty BG, et al. Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer. 2003;97(9):2142-2149.
Yang M, Long H, He J, Wang X, Xie Z. Paget’s disease of the breast: clinical analysis of 45 patients. Chinese J Clin Oncol. 2004;1(4):1672-7118.
Lui, CY., Mak, KL., Lam, HS., Chan, LK., Paget’s Disease of the Breast. J HK Coll Radiol 2003;6:42-44

Sawyer RH, Asbury DL. Mammographic appearances in Paget’s disease of the breast. Clin Radiol 1994;49:185-188.
Ikeda DM, Helvie MA, Frank TS, Chapel KL, Andersson IT. Paget disease of the nipple: radiologic-pathologic correlation. Radiology 1993;189:89-94.
Kaelin CM. Paget’s disease. In: Harris JR, ed. Diseases of the breast. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2000:677-682.
Echevarria, JJ., Lopez-Ruiz, JA., Martin, D., Imaz, I., Martin, M., Usefulness of MRI in detecting occult breast cancer associated with Paget’s disease of the nipple–areolar complex., British Journal of Radiology (2004) 77, 1036-1039.
Viehweg P, Lampe D, Buchmann J, Heywang-Kobrunner SH. In situ and minimally invasive breast cancer: morphologic and kinetic features on contrast enhancement MR imaging. Magma 2000;11:129–37.
Fu W, Mittel VK, Young SC. Paget disease of the breast: analysis of 41 patients. Am J Clin Oncol 2001;24:389–400.
Bijker N, Rutgers EJT, Duchateau L, Peterse JL, Julien JP, Cataliotti L. Breast-conserving therapy for Paget disease of the nipple. Cancer 2001;91:472–7
Paget J. On diseases of the mammary areola preceding cancer of the mammary gland. St Bartholomew Hosp Rep 1874; 10:86.