Paget's Disease Of The Nipple- Symptoms, Causes, Complications, And Treatment

Paget's (PAJ-its) disease of the breast and nipple is a rare form of breast cancer. Unlike breast cancer, cells of this kind of tumour or cancer can be observed on the nipple and a dark circle of skin called the areola. It often starts on the nipple and extends around the areola region of the nipple.

The exact cause of this breast cancer is not clear to the medical industry till now, but the test often shows a possibility that the cells from a tumor might have travelled through the milk ducts and infected the nipple and areola region. However, in many cases, women still have Paget’s disease of the nipple without any medical history or symptoms of breast tumors.

Paget's disease is commonly found in women of older age, or in the 50’s.

In Paget’s disease of the nipples, signs, and symptoms usually start in the tip of the nipple to the areola and other areas of the breast. The cancerous diseases could be easily mistaken with skin conditions like eczema and dermatitis.

The first symptom of Paget’s disease of nipples is that it is usually found on only one side of the breast, and it would not respond to any topical treatments. Apart from that here are some other symptoms that you can look for: Paget's disease of the nipples.

• Redness
• Flaking, crusting or scaling
• A tingling or itching sensation
• Thickening skin on or near your nipple
• Flattening of your nipple
• Yellowish or bloody discharge from your nipple
• Increasing breast sensitivity, soreness, and pain
• Oozing, Crusting and hardened skin. Look quite identical to eczema on the nipple and areola region.
• A lump in the breast

Also, in case of any unrecovered contusion in the breast region which does not have any sign of recovery, can also become a symptom of paget’s disease. Seek medical attention as soon as possible with the concerned doctor.

These factors develop a high-risk situation for Paget's disease of the nipples. These factors are identical to various types of breast cancer. These factors are-

1. Age:

   Chances of developing breast cancer do heighten in older age. Paget's disease of the nipple is mostly seen in 50+ women.

2. Medical history with cancer:

   If a patient had breast cancer both or one breasts, in the early stage of her life. In that case, there is an increased chance of developing Paget's cancer relapse.

3. Medical history of breast abnormalities:

   Diseases like atypical hyperplasia or lobular carcinoma in situ can increase your risk of developing breast cancer. Certain benign breast conditions can also become a risk factor.

4. Family history:

   If women or men in your family have a history with breast or ovarian cancer, the offspring will also have a greater chance of developing the same.

5. Inherited gene mutation of breast cancer:

   Defects like BRCA1 or BRCA2 are known to genetic defaults that carry a constant of developing cancer, especially in the breast.

6. Radiation exposure:

   In case a patient receives any radiation treatments in the early stages of life, especially in the chest area . It is very likely for them to develop breast cancer in older ages.

7. Obesity and Dense tissue:

   Weighing more than your ideal Body Mass Index (BMI) can make your breast tissues more dense which heightens the risk of breast cancer. Especially after menopause, where the body experiences a major downfall in regulatory hormones.

8. Hormone imbalance:

   Taking medication for estrogen imbalance after menopause can increase the risk of breast cancer in some cases.

9. Race:

   Studies have shown that there is an increased chance of developing breast cancer in white women as compared to black women yet, black women are more susceptible to die from breast cancer.

10. Smoking and drinking:

    Drinking large amounts of alcohol or smoking cigarettes for a very long time can increase the risk of developing breast cancer.

Diagnosis begins with a general physical examination. The doctor will start with observing the chest area, especially the breast, nipples and areola. The objective of this examination will be to diagnose any form of lumps or unusual thickening in the breast.Apart from physical examination, your general surgeon will advise you to run some tests.

These tests are conducted to determine the speed and spread of tumor grade, the nature of cancer (invasive or noninvasive), and if the cancer is positive for hormone receptors (HR) or HER2 gene overexpression. These tests are:

1. Mammogram:

   Is known as an X-ray examination of your breast tissue; may help your doctor to identify the underlying cause of breast cancer. One of the most common cases in Paget's disease of the nipples is underlying breast cancer. If results from mammography do not show any symptoms of cancer, the doctor will advise the patient to go for an MRI.

   MRI or Magnetic resonance imaging is a medical imaging technique used to form pictures of physiological processes of the body. It will help the doctor to examine the breasts through detailed images, which will probably identify the root cause of cancer that was not visible through mammograms.

2. Breast biopsy:

   Biopsy is a process in which the doctor will gather a small sample of tissue from the underlying skin of the breast and nipples. A sample of the nipple discharge and breast lump might also be collected, if the patient has any, For a microscopic examination. If the results show any sign of cancer cells, the patient case may shift to a breast surgeon to evaluate methods of treatment.

3. Sentinel lymph node biopsy:

   If the patient has invasive breast cancer, it is recommended to examine the udder arm skin for any spread signs. The procedure through which axillary lymph nodes get examined is known as sentinel lymph node biopsy.

During the diagnosis and treatment process, the surgeon will identify the location of the sentinel node or first lymph nodes to receive the starting point for breast tumors and therefore it will be the first place in the body where cancer cells sprouted in the breast.

If the sentinel node in the breast is successfully removed, the chances of sprouting cancer cells in any of the remaining nodes are very low and the surgeon doesn't have to remove other nodes.

Your respective oncologist will schedule a treatment plan to examine various factors, such as:

• The size and grade of the tumor
• Cancer stage
• HR and HER2 status in the breast and nipples
• Medical history
• Age and lifestyle

The method of surgery depends on the severity of the case, which includes the size and spread of cancerous cells in the breast and nipples . In the case of breast-conserving surgery, only the nipple and areola are removed, if you don’t have tumors in the breast.

If you have Paget's disease of the nipples and breast, you will be advised to have immediate surgery. The Parameters of the surgical process rely on the skin condition of the nipple and the underlying cancer stage. Surgical options include:

1. Simple mastectomy:

   This procedure involves removal of the entire breast, but not the under arms tissues or axillary lymph nodes. Simple mastectomy is recommended when breast cancer hasn't spread to the lymph nodes.

2. Lumpectomy:

   also known as Breast-conserving surgery, involves the removal of diseased portions of your breast. The surgeon may remove the nipple and areola region along with a cone-shaped section of the breast. This surgery will focus on minimal removal of breast tissue while ensuring the presence of healthy cells in the margin area.

In the case of Paget's disease, Lumpectomy will require follow-up on the radiation therapy to target any underlying pieces of cancer cells. Lumpectomy would not be recommended if the patient's body is not suitable for radiation therapy.

Also, your doctor will prescribe you a couple of powerful chemotherapy drugs to destroy any residue of cancer in any part of your body.

One potential complication in this type of breast cancer is misdiagnosed or delaying detection. Early stage cancer is easy to compare as compared to the critical ones. , but if delayed it can lead to life-threatening situations.

Treatment methods like Surgery, chemotherapy, radiation, and hormone treatments can create temporary discomforts like pain, fatigue, and skin irritation. On the other hand, these treatment methods are also known for developing a high risk for long term diseases like -

1. Lymphedema:

   lymph nodes are responsible to regulate lymph fluid in the body which prevents it from swelling. Due to the removal of lymph nodes, your body's immune system creates an imbalance which creates a blockage of lymph fluid in the body resulting in fluid buildup and swelling.

2. Chemotherapy or radiation therapy:

   It comes with its own risk, medical researchers still do not have the cure of the diseases caused due to side effects of these treatment methods. Majorly the risk includes other forms of cancers, which can also lead to early menopause or infertility.

Paget's cancer can be overwhelming, especially to your body. Many things can help a survivor to reduce your relapse of breast cancer -

1. Diet and lifestyle:

   Although there are no definite conclusions at the moment of diet balance and cancer. But a balanced form of lifestyle has always shown results in preventing and curing the surgery wound and it may help in decreasing the possibility of future cancer relapse.

   One can adapt few lifestyle changes for fast and healthy recovery:

   • Maintain a healthy body weight as per your BMI ( body mass index)
   • Lower the intake of saturated fat
   • Avoid the consumption of alcohol and tobacco
   • Increase the consumption of natural proteins and antioxidants ( especially Lycopene)
   • Add Phytochemicals to your diet ( Nutrient mostly found in plants)
   • Add Beta-carotene to your diet
   • Avoid processed and high-fat meats

   It is advisable to concern your doctor or a suggested dietician for better insight about a balanced diet.

2. Exercise:

   As the body starts to recover, the patient needs to exercise and stay physically active. Exercise is known to regulate hormones in your body, which helps in mood upliftment, fast recovery, and a healthy lifestyle.

   For the initial stages post-surgery, light physical activities like walking and stretching are recommended. Try not to work with any type of weight as it may stretch out your skin around the wound.

   Activities like yoga and meditation can also be included after consultation with your doctor. You need to avoid exercise which includes heavy arm power. This is because the lymph nodes around your arm are susceptible to swelling after surgery.

   Also, keep your wound dry and clean and avoid any kind of moisture or sweat around the operated area.

3. Breast screening and regular checkups:

   Frequent breast screening can help you detect any residue of breast cancer before it forms a lump again. As earlier, The diagnoses will take place with the help of mammograms to create an image of the inside of your breasts.