Uncommon Breast Cancers Part One: PAGET’S DISEASE OF THE NIPPLE

Teasing Fact from Fiction

OBJECTIVE:

Paget's disease of the breast is a rare form of breast cancer in which the first clinical finding is usually a scaling of the nipple skin. It may also be associated with nipple redness and burning. It can be a serious problem because 9 out of 10 women who have it have some underlying breast cancer. The underlying breast cancer may be either an invasive ductal carcinoma (IDC) or ductal carcinoma in situ (DCIS).

Paget’s also may be associated with other forms of hormonally based cancers such as uterine, ovarian or cervical. Paget’s disease is quite rare only affecting about just over 1% all diagnosed breast cancers. It is most common in women past menopause, but can infrequently occur at a younger age or in men.

The cause of Paget's disease is unknown, but certain women seem to be at a higher risk of developing breast cancer. This includes women who have never had children or had them late in life, women who started their periods at a young age or who had a late menopause, and women who have a strong family history of breast cancer. The "good news" about Paget's disease is that it is usually a "curable cancer".

However, it is important to note that, by far, most cases of scaling and burning of the nipple are due to benign conditions, which can be easily confused with Paget's disease.

Making a Diagnosis:

The major goal of this section is to help in the detection of the early nipple changes associated with Paget's disease, and to help differentiate them from benign conditions. The first symptom is usually a scaly, red rash affecting the nipple and sometimes the dark area of skin surrounding the nipple (the areola). The rash always affects the nipple first, and may then affect the areola. It doesn't go away and may become sore. The skin of the nipple and areola may be inflamed. There may also be crusting, bleeding and ulceration. Some women have an itching or burning sensation. Fluid (discharge) may leak from the abnormal area of cells. The nipple may turn inwards (be inverted). There may or may not be a lump in the breast.

Paget’s disease can be confused with other skin conditions such as eczema, dermatitis or psoriasis, as they can look very similar. This can make Paget’s disease difficult to diagnose with BSE or CBE. Paget’s disease usually affects the nipple first and then the surrounding tissue. Other skin conditions usually affect the areola (the dark area of skin around the nipple) first, and then spread into the nipple.

The first step in evaluating any patient with a change in the skin of the nipple is to obtain a careful history and perform a careful breast examination. A diagnostic mammogram, as opposed to a screening study should follow. When performing the diagnostic mammogram, the Mammographer, following up the possibility of Paget's disease, should acquire coned magnification films of the retro nipple area to look for fine calcifications. A U/S of the breast is also a useful tool in the diagnosis of underlying breast cancer often associated with Paget’s disease. Moreover, because of the possibility of other hormonally based cancers (uterine, ovarian etc.) a U/S evaluation of the pelvis is sometimes recommended.

If the mammogram and U/S are negative and the clinical suspicion is low, it is usually suggested that the patient be placed on cortisone cream treatment applied to the nipple for two weeks. If the condition has not responded completely to the conservative treatment in two weeks, a skin biopsy should be performed. If, because of negative imaging results and good clinical results, a biopsy is not done, careful follow-up is highly recommended.

Making an accurate diagnosis of Paget's disease is actually quite easy. A simple skin tissue biopsy to remove a small piece of tissue from the area of the nipple affected by the abnormal changes is performed. This procedure takes only a few minutes to complete under local anaesthesia, and is painless. A small amount of skin and underlying tissue are removed and sent to the pathologist; these biopsies result in a 100% definitive diagnosis.

Treatment of Paget’s disease:
In the past, the standard treatment for malignant Paget's disease was a mastectomy. Currently, the treatment of choice is to save the breast. The nipple and any surrounding breast tissue that contains breast cancer are removed. If there are definitive clear surgical margins, then irradiation of the affected breast follows. The results with this approach have been excellent. In some cases, there will be more extensive involvement of the breast tissue or an underlying cancer is diagnosed. In these cases, a mastectomy is recommended usually followed by immediate reconstruction.

Images of Paget’s Disease of the Nipple

Figure 1: Above, Normal Right Nipple & below, Biopsy proven Paget's Left Nipple

Figure 2: Very Early Paget's disease

Figure 3: Advanced Paget's disease of the Nipple

Figure 4: Very Advanced Paget's disease of the left nipple

Benign Conditions of the Nipple:
Many of the women referred with a possible Paget's disease have similar changes in both nipples. There is not a documented case of Paget’s affecting both breasts; therefore, most such patients are referred to a dermatologist to evaluate the underlying cause of the nipple change. However, it would be prudent to insist that these patients have a mammogram to rule out any breast involvement that is not clinically obvious. If there are any reasons for concern about the possibility of Paget's disease diagnosis, a skin punch biopsy should be performed.

Most patients who do have a skin punch biopsy end up with a benign result. These patients simply require reassurance that there is no cancer present, and follow-up observation is all that is indicated. Sometimes a moisturizing cream is helpful to control any nipple dryness. Patients with persistent symptoms following a negative biopsy are also referred to a dermatologist for treatment.

There are a variety of conditions that can mimic Paget's disease, such as atopic dermatitis or nipple eczema, or contact dermatitis. Some Images of common benign nipple conditions follow:

Benign Conditions Affecting the Nipple:

Figure 5: Normal Prominent Montgomery Tubercles

Figure 6: Contact Dermatitis

Figure 7: Right Dermal Cyst. Left is a close-up view

Figure 8: Above: Spongiotic Dermatitis left nipple. Below: Normal right nipple

SUMMARY:
We are the ‘thin pink line’ that stands as a protection against ignorance, a source of pertinent breast health information and as a foundation for the start of treatment or diagnosis. We have the chance to be the voice of reason, to dispel myth and present fact. The more we know about signs and symptoms that affect our patients the better professionals we become.

Power is knowledge; the truth is much less scary than myth, story and insinuation.