Paget Disease of the Nipple: Questions and Answers

I found this fact sheet and wanted to pass the information along to everyone. It has a lot of great information.

Paget Disease of the Nipple: Questions and Answers

Key Points
• Paget disease of the nipple is an uncommon type of cancer that forms in or around the nipple (see Question 1).
• Paget disease of the nipple is almost always associated with an underlying breast cancer (see Questions 1 and 2).
• Scientists do not know exactly what causes Paget disease of the nipple, but two major theories have been suggested for how it develops (see Question 2).
• Symptoms of early-stage disease may include redness or crusting of the nipple skin; symptoms of more advanced disease often include tingling, itching, increased sensitivity, burning, or pain in the nipple (see Question 3).
• Paget disease of the nipple is diagnosed by performing a biopsy (see Question 4).
• Surgery is the usual treatment for Paget disease of the nipple. Additional treatments may be recommended under certain circumstances (see Question 5).
• Many clinical trials for breast cancer are under way (see Question 6).


1. 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.

2. 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).

3. 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).

4. 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.

5. 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).

6. 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.


Selected References
1. Kaelin CM. Paget's Disease. In: Harris JR, Lippman ME, Morrow M, Osborne CK, editors. Diseases of the Breast. 3rd ed. Philadelphia: Lippincott Williams and Wilkins, 2004.
2. DeVita,VT Jr., Hellman S, Rosenberg SA, editors. Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia: Lippincott Williams and Wilkins, 2004.
3. Beers MH, Berkow R, editors. The Merck Manual of Diagnosis and Therapy. 17th ed. Whitehouse Station, NJ: Merck & Company, Inc., 1999.
4. American Cancer Society (2005). Cancer Facts and Figures 2005. Atlanta, GA: American Cancer Society. Retrieved April 20, 2005, from http://www.cancer.org/downloads/STT/CAFF2005f4PWSecured.pdf.
5. Marcus E. The management of Paget's disease of the breast. Current Treatment Options in Oncology 2004; 5:153–160.

Related NCI materials and Web pages:
• National Cancer Institute Fact Sheet 2.11, Clinical Trials: Questions and Answers (http://www.cancer.gov/cancertopics/factsheet/Information/clinical-trials)
• National Cancer Institute Fact Sheet 5.14, Improving Methods for Breast Cancer Detection and Diagnosis (http://www.cancer.gov/cancertopics/factsheet/Detection/breast-cancer)
• National Cancer Institute Fact Sheet 7.1, Radiation Therapy for Cancer: Questions and Answers (http://www.cancer.gov/cancertopics/factsheet/Therapy/radiation)
• National Cancer Institute Fact Sheet 7.20, Adjuvant Therapy for Breast Cancer: Questions and Answers (http://www.cancer.gov/cancertopics/factsheet/Therapy/adjuvant-breast)
• Surgery Choices for Women With Early-Stage Breast Cancer (http://www.cancer.gov/cancertopics/breast-cancer-surgery-choices)
• Understanding Breast Changes: A Health Guide for Women (http://www.cancer.gov/cancertopics/understanding-breast-changes)
• What You Need To Know About™ Breast Cancer (http://www.cancer.gov/cancertopics/wyntk/breast)

This fact sheet was reviewed on 6/27/05

Good Health to us all and a Happy 4th of July tomorrow!!

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Farrah Fawcett Loses her Battle with Cancer

Last Thursday Farrah Fawcett lost her Battle with Cancer. Her death was somewhat over shadowed by the death of Michael Jackson.

Farrahs battle with cancer was made public in 2006. She created a show that followed her on her tireless rounds of treatments from the disease. This story "Farrah's Story" recently was aired on television.

The show showed the up and downs with dealing with chemotherapy, hair loss, depression and the hopes and fears of the unknown future.

Farrah's story also showed the strength she had and the love her family and friends and how they helped give her more strength.

All cancer survivors have to go through the same, whether for a short time or over years. We try to live our lives showing the same strength and appreciate all the love and support we get from our family and friends.

Farrah put it all in front of the TV audience for everyone to see what a deadly word CANCER really is. This helps other people understand and comprehend just how devastating hearing those words "you have cancer" can be and the fight it takes not to let it defeat you. You just keep battling until the end.

Farrah Fawcett fought a brave battle and her story will help others along the way.

Thank you, Farrah, for allowing us to see your strength.

Good health to us all, and say a prayer for Farrah. Leigh

AVON Vintage Ivory Color Bracelet and Earring Set

As many of you know I am selling items in eBay to help pay my medical bills which have accummulated since my diagnosis of Breast Cancer. I have no insurance and did not qualify for the breast cancer/cervical cancer program in my state. My cancer was not diagnoised at the county health department but at my doctor's office which according to the state made it a pre-existing condition thus eliminating my having access to their program. This is why I am resposible for all the bills. But I decided at the time that there was still a lot I had to do with my life and it was worth whatever I needed to do to keep going.

The reason for this little message is that occasionally I will be showcasing an item I have for sale at eBay in my Blog. I have Vintage jewelry, glass, books, clothes and more. I hope you do not mind but I wanted to get some extra exposure for the items. I hope you will look and see if there is anything you might like for yourself or as a gift for someone you know. I appreciate all the help and support I have recieved from my readers and from my eBay customers. Thank you!

FOR SALE! Vintage AVON Ivory Color Bracelet and Earring Set

This set was made in Japan, Exclusively for Avon. I do not know if it is Ivory or Shell. It does not look like plastic. Elastic in the bracelet and Clip Earrings.

You can go to this auction by clicking Here

Thanks again for you help, Leigh

PARP Inihibitors, Breast Cancer, Years of Research Ahead?

PARP Inhibitors. PARP is short for "poly (ADP-ribose) polymerase," which is used by cancer cells to repair DNA damage, including the damage inflicted by chemotherapy drugs. By blocking the enzyme, researchers aim to further undermine the ability of cancer cells to heal themselves.

Yesterday it was announced that these PARP Inhibitors may be the greatest breakthrough for breast cancer patients since the development of Herceptin in the late 1990's.

According to the investigators in the most recent string of favorable studies almost two-thirds of mutation carriers had a clinical benefit from treatment with olaparib, Johann S. de Bono, MD, PhD, of the Royal Marsden Hospital in London, and colleagues reported in the June 24 issue of the New England Journal of Medicine

I hope they are right. 15% to 20% of all women with breast cancer have the BRCA1/2 mutation. This drug has also shown favorable results in test of women with triple-negative breast cancer (as stated in May at the annual meeting of the American Society of Clinical Oncology.)

The agent demonstrated activity in patients with breast, ovarian, and prostate cancer.

The following note was attached to one of the articles I found on google. Action Points for Doctors to tell Patients.

Action Points --->
1. Explain to patients that an investigational drug showed activity in tumors that have certain types of genetic mutations.
2.Emphasize that the drug is not yet available.

My one hope is that "The Powers that Be" will allow testing to continue at a rate that approval can be given quickly for this medication to get to the people who need it.

Great Health and Long Life to us all, Leigh

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Inflammatory Breast Cancer Fact Sheet from the National Cancer Institute.

I found this Fact Sheet and it answered many question I had not been able to answer before.

Hope you find it as informative as I did.

_____________________________________________________________

Inflammatory Breast Cancer: Questions and Answers

1. What is inflammatory breast cancer (IBC)?

Inflammatory breast cancer is a rare but very aggressive type of breast cancer in which the cancer cells block the lymph vessels in the skin of the breast. This type of breast cancer is called "inflammatory" because the breast often looks swollen and red, or "inflamed." IBC accounts for 1 to 5 percent of all breast cancer cases in the United States (1). It tends to be diagnosed in younger women compared to non-IBC breast cancer. It occurs more frequently and at a younger age in African Americans than in Whites. Like other types of breast cancer, IBC can occur in men, but usually at an older age than in women. Some studies have shown an association between family history of breast cancer and IBC, but more studies are needed to draw firm conclusions (2).

Key Points

• Inflammatory breast cancer (IBC) is a rare but very aggressive type of breast cancer (see Question 1).
• IBC usually grows rapidly and often spreads to other parts of the body; symptoms include redness, swelling, and warmth in the breast (see Questions 2 and 3).
• Treatment for IBC usually starts with chemotherapy, which is generally followed by surgery, radiation, targeted therapy, and/or hormone therapy (see Question 4).
• People with IBC are encouraged to enroll in clinical trials (research studies with people) that explore new treatments (see Question 5).

2. What are the symptoms of IBC?

Symptoms of IBC may include redness, swelling, and warmth in the breast, often without a distinct lump in the breast. The redness and warmth are caused by cancer cells blocking the lymph vessels in the skin. The skin of the breast may also appear pink,
reddish purple, or bruised. The skin may also have ridges or appear pitted, like the skin of an orange (called peau d'orange), which is caused by a buildup of fluid and edema
(swelling) in the breast. Other symptoms include heaviness, burning, aching, increase in breast size, tenderness, or a nipple that is inverted (facing inward) (3). These symptoms usually develop quickly—over a period of weeks or months. Swollen lymph nodes may also be present under the arm, above the collarbone, or in both places. However, it is important to note that these symptoms may also be signs of other conditions such as infection, injury, or other types of cancer (1).

3. How is IBC diagnosed?

Diagnosis of IBC is based primarily on the results of a doctor’s clinical examination (1). Biopsy, mammogram, and breast ultrasound are used to confirm the diagnosis. IBC is classified as either stage IIIB or stage IV breast cancer (2). Stage IIIB breast cancers are locally advanced; stage IV breast cancer is cancer that has spread to other organs. IBC tends to grow rapidly, and the physical appearance of the breast of patients with IBC is different from that of patients with other stage III breast cancers. IBC is an especially aggressive, locally advanced breast cancer.
Cancer staging describes the extent or severity of an individual’s cancer. (More information on staging is available in the National Cancer Institute (NCI) fact sheet Staging: Questions and Answers at http://www.cancer.gov/cancertopics/factsheet/Detection/staging on the Internet.) Knowing a cancer’s stage helps the doctor develop a treatment plan and estimate prognosis (the likely outcome or course of the disease; the chance of recovery or recurrence).

4. How is IBC treated?

Treatment consisting of chemotherapy, targeted therapy, surgery, radiation therapy, and hormonal therapy is used to treat IBC. Patients may also receive supportive care to help manage the side effects of the cancer and its treatment. Chemotherapy (anticancer drugs) is generally the first treatment for patients with IBC, and is called neoadjuvant therapy. Chemotherapy is systemic treatment, which means that it affects cells throughout the body. The purpose of chemotherapy is to control or kill cancer cells, including those that may have spread to other parts of the body.

After chemotherapy, patients with IBC may undergo surgery and radiation therapy to the chest wall. Both radiation and surgery are local treatments that affect only cells in the tumor and the immediately surrounding area. The purpose of surgery is to remove the tumor from the body, while the purpose of radiation therapy is to destroy remaining cancer cells. Surgery to remove the breast (or as much of the breast tissue as possible) is called a mastectomy. Lymph node dissection (removal of the lymph nodes in the underarm area for examination under a microscope) is also done during this surgery.

After initial systemic and local treatment, patients with IBC may receive additional systemic treatments to reduce the risk of recurrence (cancer coming back). Such treatments may include additional chemotherapy, hormonal therapy (treatment that interferes with the effects of the female hormone estrogen, which can promote the growth of breast cancer cells), targeted therapy (such as trastuzumab, also known as Herceptin®), or all three. Trastuzumab is administered to patients whose tumors overexpress the HER–2 tumor protein. More information about Herceptin and the HER–2 protein is available in the NCI fact sheet Herceptin® (Trastuzumab): Questions and Answers, which can be found at http://www.cancer.gov/cancertopics/factsheet/therapy/herceptin on the Internet.

Supportive care is treatment given to improve the quality of life of patients who have a serious or life-threatening disease, such as cancer. It prevents or treats as early as possible the symptoms of the disease, side effects caused by treatment of the disease, and psychological, social, and spiritual problems related to the disease or its treatment. For example, compression garments may be used to treat lymphedema (swelling caused by excess fluid buildup) resulting from radiation therapy or the removal of lymph nodes. Additionally, meeting with a social worker, counselor, or member of the clergy can be helpful to those who want to talk about their feelings or discuss their concerns. A social worker can often suggest resources for help with recovery, emotional support, financial aid, transportation, or home care.

5. Are clinical trials (research studies with people) available? Where can people get more information about clinical trials?

Yes. The NCI is sponsoring clinical trials that are designed to find new treatments and better ways to use current treatments. Before any new treatment can be recommended for general use, doctors conduct clinical trials to find out whether the treatment is safe for patients and effective against the disease. Participation in clinical trials is a treatment option for many patients with IBC, and all patients with IBC are encouraged to consider treatment in a clinical trial.

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 is available 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 comprehensive cancer information database. The CIS also provides information from PDQ.

6. What is the prognosis for patients with IBC?

Prognosis describes the likely course and outcome of a disease—that is, the chance that a patient will recover or have a recurrence. IBC is more likely to have metastasized (spread to other areas of the body) at the time of diagnosis than non-IBC cases (3). As a result, the 5-year survival rate for patients with IBC is between 25 and 50 percent, which is significantly lower than the survival rate for patients with non-IBC breast cancer. It is important to keep in mind, however, that these statistics are averages based on large numbers of patients. Statistics cannot be used to predict what will happen to a particular patient because each person’s situation is unique. Patients are encouraged to talk to their doctors about their prognosis given their particular situation.

7. Where can a person find more information about breast cancer and its treatment?

To learn more about IBC, other types of breast cancer, and breast health in general, please refer to the following resources:

• NCI’s Breast Cancer Home Page (http://www.cancer.gov/breast/)
• Breast Cancer (PDQ®): Treatment (http://www.cancer.gov/cancertopics/pdq/treatment/breast/patient/)
• Understanding Breast Changes: A Health Guide for All Women (http://www.cancer.gov/cancertopics/understanding-breast-changes)
• What You Need To Know About™ Breast Cancer (http://www.cancer.gov/cancertopics/wyntk/breast)

Selected References

1. Merajver SD, Sabel MS. Inflammatory breast cancer. In: Harris JR, Lippman ME, Morrow M, Osborne CK, editors. Diseases of the Breast. 3rd ed. Philadelphia: Lippincott Williams and Wilkins, 2004.
2. Anderson W, Schairer C, Chen B, Hance K, Levine P. Epidemiology of inflammatory breast cancer (IBC). Breast Disease 2005; 22:9–23.
3. Chittoor SR, Swain SM. Locally advanced breast cancer: Role of medical oncology. In: Bland KI, Copeland EM, editors. The Breast: Comprehensive Management of Benign and Malignant Diseases. Vol. 2. 2nd ed. Philadelphia: W.B. Saunders Company, 1998.

# # #

Related NCI materials and Web pages:
• National Cancer Institute Fact Sheet 2.1, Cancer Information Sources
(http://www.cancer.gov/cancertopics/factsheet/Information/sources)
• National Cancer Institute Fact Sheet 5.32, Staging: Questions and Answers
(http://www.cancer.gov/cancertopics/factsheet/Detection/staging)
• National Cancer Institute Fact Sheet 7.1, Radiation Therapy for Cancer: Questions and Answers (http://www.cancer.gov/cancertopics/factsheet/Therapy/radiation )
• National Cancer Institute Fact Sheet 7.2, Biological Therapies for Cancer: Questions and Answers (http://www.cancer.gov/cancertopics/factsheet/Therapy/biological)
• National Cancer Institute Fact Sheet 7.45, Herceptin® (Trastuzumab): Questions and Answers (http://www.cancer.gov/cancertopics/factsheet/therapy/herceptin)
• Chemotherapy and You: Support for People With Cancer (http://www.cancer.gov/cancertopics/chemotherapy-and-you)
• Taking Part in Cancer Treatment Research Studies
(http://www.cancer.gov/clinicaltrials/Taking-Part-in-Cancer-Treatment-Research-Studies)

For more help, contact:

NCI’s Cancer Information Service
Telephone (toll-free): 1–800–4–CANCER (1–800–422–6237)
TTY (toll-free): 1–800–332–8615
LiveHelp® online chat: https://cissecure.nci.nih.gov/livehelp/welcome.asp
This fact sheet was reviewed on 8/29/06

Good Exercise is Important for Cancer Health! Try Golf!

One of the things my doctors told me during my fight with cancer is to try to stay active. They said this would help with depression and muscle aches.

Well, I did not because I felt so bad and was that a mistake. I had no energy, felt washed out, and generally felt like crap. Sorry about the language but that was how I felt.

My sister asked me one day to come out and play some golf with her. She had just had gastric bypass surgery and needed to walk everyday. She hated just walking down the block and she used to play golf occasionally so she thought she could get her exercise that way.

I had never played golf and really did not care to learn but I wanted to support her in any way that I could so I said OK.

Well, I found out something out there on the golf course. It made me feel better to get out of the house and walk around and get some fresh air.

Now I may not be Tiger Woods, but my sister and I both are enjoying getting a little exercise and sunshine. Yes we take our time, drink lots of water and we don't do 18 holes. But who cares, we are having a good time and feeling better.

Since I don't know a lot about golf I decided to check out some of the information on the web and found this great site called http://www.golf.todays-tips.com/ and they have some great information. They have the basics for people like me and videos for improving your swing, curing your slice, chip shots and my favorite, getting out of the bunker. The also have information on Golf health, Golf Vacations and Golf Equipment.

I am improving every time we play and I have two ladies joining us this week from my cancer support group. They thought it sounded like lots of fun and wanted to try.

Ladies, whether it is golf, tennis, swimming or just walking around the block, Get out of the House! If you are feeling bad, it will make you feel better. I wear my wide brimmed hat and lots of sunscreen, light weight clothing and get out there and go.

Don't worry about your score, remember it is just a game and HAVE FUN!!!!

Health and Happiness to everyone, Leigh

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