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Classification/Stages In Paget’s Disease Of The Breast

Paget’s disease of the breast, a rare type of cancer that outwardly may have the appearance of eczema is classified as mammary and extramammary.1

A review including clinical, pathological, and immunohistochemical findings demonstrates skin nodules were classified as T4b because of their dire prognosis and lack of benefit from surgery.2

In the early stages, most cases of Paget’s Disease of the breast do not produce any specific symptoms.3,4

Paget disease is a rare skin manifestation of breast cancer and often associated with intraductal cancer. It occurs when there is an infiltration of the nipple epidermis by adenocarcinoma cells.

The condition initially shows as changes to the nipple and prominently noticed in less than 5% of all women with breast melanomas. Nearly 1 to 4 in every 100 breast cancer cases is this type of disease.

Classification/Stages In Paget’s Disease Of The Breast

Studies show that patients with direct dermal invasion from Paget disease of the breast had a favorable outcome when the condition was diagnosed during the initial onset of the condition. On the contrary, there are Paget’s patients showing no symptoms at all therefore, clinical findings in such cases become challenging.

Paget cells are typically found in the nipple according to histopathologic examination, usually at mastectomy and they exhibit as a lump behind the nipple area or bloody or yellowish discharge from the nipple. The symptoms are often diagnosed during imaging examinations.

Research states that patients who exhibit changes in the appearance of the nipple had less extensive cancers when compared to the patients who were clinically identified at mastectomy.1

Paget’s disease of the mammary glands includes a range of locations and the diagnosis is dependent on certain additional measures that include colonoscopy, mammography, cervical cytology, Papanicolaou staining, and colposcopy.

Although biopsy is typically performed to confirm the presence of malignancy, additional diagnosis is carried out to find the extent of the disease. 2. A review including clinical, pathological, and immunohistochemical findings demonstrates skin nodules were classified as T4b because of their dire prognosis and lack of benefit from surgery.

However, according to the Wilkinson and Brown classification, Paget’s disease is classified as:

Primary Of The Cutaneous Origin – This condition occurs within the epidermis or the underlying apocrine glands. It represents 6.5% of all cutaneous Paget disease and mainly affects patients between the ages group of 50 to 80 years of age with majorly 65.3 years old. Although the condition is limited to the epithelium, yet it has the potential to grow and spread to the other parts of the body resulting as an invasive tumor making the condition severe and necessitating high-density treatments. The primary condition is further divided into the following types

  • Usual type
  • Invasive
  • A characteristic of underlying skin neoplasm and turns into a disease of the vulva.

Secondary Of The Non-Cutaneous Origin- In contrast to the cutaneous origin, the non-cutaneous malignancy is often associated with underlying adnexal or visceral carcinoma. The standard management strategy of this condition is usually through surgery however clinical studies demonstrate that the recurrence rate is very high. This is sub-classified into

  • Anorectal origin
  • Urothelial origin
  • Another origin

In the initial stages, there is a high frequency of associated malignancy and these patients show poor outcomes requiring an extensive search for the secondary malignancy. However, during stage 2, there is a spread of the underlying secondary carcinoma and is treated by neoadjuvant and adjuvant chemotherapy.3,4


  1. Lopes Filho, Lauro Lourival, et al. “Mammary and Extramammary Paget’s Disease.” Anais Brasileiros De Dermatologia, Sociedade Brasileira De Dermatologia, 2015, www.ncbi.nlm.nih.gov/pmc/articles/PMC4371672/.
  2. Thomas, Liji. “Extramammary Paget’s Disease Classification and Diagnosis.” News, 27 Feb. 2019, www.news-medical.net/health/Extramammary-Pagets-Disease-Classification-and-Diagnosis.aspx.
  3. “Paget Disease of the Breast.” National Cancer Institute, www.cancer.gov/types/breast/paget-breast-fact-sheet.
  4. “Paget Disease of the Breast – Canadian Cancer Society.” Www.cancer.ca, www.cancer.ca/en/cancer-information/cancer-type/breast/breast-cancer/cancerous-tumours/paget-disease-of-the-breast/?region=on.

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Team PainAssist
Team PainAssist
Written, Edited or Reviewed By: Team PainAssist, Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:June 18, 2020

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