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Paget’s Disease of the Nipple

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Updated May 16, 2014

What is Paget’s disease of the nipple?:

Paget’s disease of the nipple or breast is a rare type of breast cancer, which can occur in women and men. It shows up in and around the nipple, and usually signals the presence of breast cancer beneath the skin. Most cases are found in menopausal women, but can also appear in women that are as young as 20. The disease is named for Sir James Paget. He reported on the link between changes in the nipple and the underlying breast cancer.

Signs and symptoms:

Early stages: (appearance of nipple skin)
  • redness
  • scaly and flaky skin
  • mild irritation of skin
Advanced stages:
  • tingling in nipple skin
  • itchiness that doesn’t respond to creams
  • very sensitive skin on the nipple
  • burning or painful nipple skin
  • ooze or bloody discharge from the nipple (not milk)
  • nipple retraction (pulls into the breast)
  • scaly rash on areola skin
  • breast lump beneath the affected skin

Tests to diagnose Paget's Disease of the nipple:

If any of the signs and symptoms appear and do not respond to home treatment (creams or ointments), see a health professional. The following tests can be done to pinpoint what the cause may be.
  • visual examination
  • biopsy and pathology of tissue sample
  • analysis of nipple discharge
  • mammography and/or ultrasound
  • breast MRI

What may cause Paget’s disease of the nipple?:

Research is still being done on the cause of Paget’s disease, but there are two leading theories.

(1) If invasive breast cancer or ductal carcinoma in situ (DCIS) is already present in the breast as a tumor, cells might drift off from the tumor and float up through the milk ducts, where they enter the nipple and areola.

(2) In a few cases of Paget’s disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. Researchers suggest that in those cases, nipple skin cells may spontaneously change into cancer cells.

Treatments:

Each case of Paget’s disease will need treatment to match the size and stage of the cancer. The most common treatments are: Surgery
  • modified radical mastectomy
  • simple mastectomy
  • lumpectomy
  • sentinel lymph node biopsy (to determine whether or not the cancer has spread beyond the original site)
Adjuvant Treatments
  • radiation (to prevent return of the cancer)
  • chemotherapy (depends on the stage of the cancer)
  • hormone therapy

About Sir James Paget:

Paget lived from 1814-1889 and was a surgeon in Victorian England. He was apprenticed to a surgeon at age 16, and became a licensed surgeon at age 22. Paget taught himself anatomy by doing postmortem exams, and learned German on his own, so that he could read the medical publications from the great anatomists and surgeons of that day. He published many articles on his research, which included Paget's disease of the nipple and Paget's disease of the bone, and he discovered the parasitic worm that causes trichinosis. Paget was married for 50 years to his wife Lydia, and their son became the Bishop of Oxford.

References:

Paget’s Disease of the Nipple: Questions and Answers. Cancer Topics. National Cancer Institute. Last Updated: 6/27/05. Paget’s Disease of the Nipple: Questions and Answers

Diagnosis and Treatment of Paget’s disease of the Nipple. Stanford Cancer Center. Last updated: (not known). Diagnosis and Treatment of Paget’s disease of the Nipple

Sir James Paget (1814-1889). Famous Surgeons. Surgical-Tutor.org.uk. Last updated: (not known) Sir James Paget
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  5. Nipple and Areola Health
  6. Paget’s Disease of the Nipple - Breast Cancer

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