Paget’s disease of the breast
Paget’s disease is specific kind a neoplastic (new cell) growth which begins with the nipple, and gradually spreads to other areas of the areola and beyond.
Please note. This page is old, and a new section about Paget’s disease is here, but it’s too short, so this page is still useful.
Paget’s disease 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 now called ‘Paget’s disease of the bone‘, but these two conditions which bear his name are completely unrelated medically.
Paget’s disease can be mistaken for a mild skin irritation
Initially, Paget’s disease might resemble a benign skin condition like eczema and contact dermatitis, or other benign breast neoplasms such as duct ectasia or nipple adenoma, so there is some danger that it might be dismissed and finally discovered at a more advanced stage.
Paget’s disease is considered a form of breast cancer, and 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.
Symptoms of Paget’s disease of the breast.
Paget’s disease causes several ‘inflammatory‘ skin and nipple changes, but the most common symptom of Paget’s disease is bloody nipple discharge.
Pain and itching are also common. Some women describe their symptoms as a ‘wound which won’t heal‘ (ulceration) There might also be ‘scaling‘ of the skin, erythema (red rash) and even nipple retraction 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 with Paget’s breast disease will present with a palpable breast ‘lump‘. At the same time, of those women without any obvious breast lumps, about 50% will not show a mass within the breast on a mammographic screening.
So if there is no palpable lump, no mass revealed on mammogram, and no obvious or destructive nipple changes, it is easy to see how Paget’s disease can often be too-easily dismissed and misdiagnosed, especially if it occurs in both breasts (bilaterally). All patients with uncertain clinical symptoms of Paget’s disease should undergo mammography followed by a biopsy to confirm or exclude the diagnosis.
Histological Evaluation and diagnosis of Paget’s breast disease: Paget’s cells.
Since Paget’s disease is essentially an infiltration of the nipple-areola complex epidermis (outer skin layers) by adenocarcinoma cells (derived from glandular epithelium), 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, and 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‘ in 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 a tumor is discovered 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. Paget’s disease, once fully diagnosed, tends to be treated in a highly aggressive fashion. Magnetic resonance imaging (MRI) tends not to be used for Paget’s disease evaluation due to its non-specificity. MRI is useful in the detecting occult (hidden) breast neoplasia, but not all Paget’s disease cases will feature cell neoplasia.
Really, Paget’s disease has to be diagnosed by looking at the cells directly. An experienced radiologist, however, might be able to detect small changes to the inner structure of the nipple-areolar complex, which may cause suspicions of Paget’s disease and make the case for a fully histological evaluation.
Treatment of Paget’s disease of the Breast
Paget’s disease is considered a serious and aggressive form of breast cancer and the first line of treatment is usually surgery. Up until recently the tendency has been 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. It is now understood that when a lump has developed 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 required. However, this has to be balanced against the unfortunate tendency for Paget’s disease to have a high rate of local recurrence.
Patients are often treated by a conservative surgical excision
For patients with a palpable lump, a more conservative ‘surgical excision‘ approach is sometimes adopted. It is very important that wide, clear margins be achieved. Surgical excision is usually followed up by tissue sampling from adjacent quadrants and axillary node dissection. Some patients who have no lump and a normal mammogram have been treated by excision of the nipple-areola complex alone. This approach is typically combined with post-operative 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.
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 80-90%. 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 survival rate for patients with invasive breast cancer with or without Paget’s disease.
A reminder about this page being 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 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 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 condition. Possible signs and symptoms may include flaky or scaly skin on your nipple; crusty, oozing or hardened skin resembling eczema on the nipple, areola or both; itching; redness; a tingling or burning sensation; straw-colored or bloody nipple discharge; a flattened or inverted nipple; a lump in the breast; or 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 an underlying ductal breast cancer. The cancer cells from the original tumor then travels through the milk ducts to the nipple and its 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 your age, a personal history of breast cancer, a personal history of breast abnormalities, your family history, genetic predisposition, dense breast tissue, radiation exposure, excess weight, hormone replacement, or your 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 be aware of any pre-appointment restrictions, write down any symptoms you may be experiencing, write down 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, and 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, and 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, and 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, getting enough exercise, limiting alcohol, eating healthy foods, trying to maintain a healthy diet and healthy weight. Some interventions may help reduce your chance as well for example preventive medications, and risk-reducing surgery.
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