Paget's disease of the breast | |
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Other names | Paget disease of the breast, Paget's disease of the nipple, Mammary Paget's disease [1] |
Paget's disease of the nipple | |
Pronunciation |
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Specialty | Oncology |
Named after | James Paget |
Paget's disease of the breast (also known as mammary Paget's disease) is a rare skin change at the nipple nearly always associated with underlying breast cancer. [2] Paget's disease of the breast was first described by Sir James Paget in 1874. [3] The condition is an uncommon disease accounting for 1 to 4% of all breast cancers cases. [2] 92% to 100% of patients with Paget's disease of the breast have an underlying breast cancer. [4]
The condition in itself often appears innocuous, limited to a surface appearance and it is sometimes dismissed, although it is actually indicative of underlying breast cancer. [3] [5]
Paget's disease of the breast can affect the nipple and areola: the nipple is typically affected first and then the skin changes spread to the areola. It is common for symptoms to wax and wane. Symptoms typically only affect one breast and may include:
A person with Paget's disease of the breast may experience signs and symptoms for several months before a diagnosis is made. [7]
Paget's disease of the breast is characterized by Paget cells: large cells with clear cytoplasm (clear halo) and eccentric (not centered), hyperchromic (darkly colored) nuclei found throughout the epidermis. [5] [8]
There is some controversy as to whether these cancer cells travel through the ductal system of the breast to the nipple, [9] or whether these cells result from in situ malignant transformation. [2]
The most widely accepted theory of how Paget's disease of the breast arises is the migratory theory: ductal carcinoma in situ cancerous cells migrate into the lactiferous sinuses and out of the nipple onto the skin. [2] Cancer cells disrupt the normal epithelial barrier and fluid accumulates on the surface of the skin, resulting in the crusting of the skin of the nipple and/ or areola. [9]
During a physical examination, the provider will likely conduct a breast examination: evaluating the appearance of the skin on and around the nipples, and feeling for any lumps or areas of thickening in the breast and armpit. [10]
Paget's disease of the breast is difficult to diagnose by physical exam alone due to its resemblance to dermatitis and eczema. One helpful differentiator is that eczema tends to affect the areola first, and then the nipple, whereas Paget's disease of the breast typically begins at the nipple and spreads outwards. In addition, nipple eczema is typically responsive to topical steroid application, while Paget's disease of the breast will not improve with topical steroid use. [4]
Mammogram and biopsy with cytopathology are common confirmatory tests. [11] In biopsy, a tissue sample removed from the affected area is then examined under the microscope by a pathologist, who distinguishes Paget cells from other cell types by staining tissues to identify specific cells (immunohistochemistry). [5]
Less commonly, samples of nipple discharge may also be examined under the microscope to determine whether Paget cells are present. [6] In addition, ultrasound of the breast may be performed in the absence of positive findings on mammography. [4]
Paget's disease of the breast is a symptom of underlying breast cancer. Treatment is variable and is determined by the type of breast cancer in addition to its staging and prognostic considerations. Management often involves a lumpectomy or mastectomy to surgically remove the tumour. [12] Chemotherapy and/or radiotherapy may also be necessary. [13]
Patients with Paget's disease of the breast that has not spread beyond the nipple are often treated with breast-conserving surgery: removal of the cancerous area of the nipple and areola, but conservation of the rest of the breast. Patients then usually undergo radiation therapy after surgery as an adjuvant treatment to prevent recurrence. [13]
In most cases, adjuvant treatment is part of the treatment schema. Adjuvant therapy is given to patients with cancer as a secondary form of treatment to minimize the risk of recurrence by targeting undetectable metastases. Whether adjuvant therapy is needed depends upon the type of cancer and its staging. In Paget's disease of the breast, the most common type of adjuvant therapy is radiation following breast-conservative surgery as discussed above. [13]
Paget's disease of the breast with underlying breast cancer is primarily treated with mastectomy. In cases of invasive cancer, radical mastectomy is performed: removal of the breast, the lining over the chest muscles, and affected lymph nodes from under the arm. In cases of noninvasive cancers, simple mastectomy are performed in which only the breast with the lining over the chest muscles is removed. [13]
Three factors are evaluated when determining prognosis for breast cancer: whether there is a palpable mass, whether lymph nodes have cancer cells in them, and whether there is an underlying metastatic cancer. Prognosis of Paget's disease of the breast with underlying breast cancer depends on these three factors of the underlying cancer. Whether or not a patient has Paget's disease of the breast does not affect their prognosis in the presence of underlying breast cancer. [9]
Patients with Paget's disease of the breast and no underlying breast cancer have a 5-year relative survival rate of 82.6%. [2]
Most patients diagnosed with Paget's disease of the breast are over age 50, but rare cases have been diagnosed in patients in their 20s. The average age at diagnosis is 57. The disease is rare among both women and men, but more common in women. [2]
Breast reconstruction is the surgical process of rebuilding the shape and look of a breast, most commonly in women who have had surgery to treat breast cancer. It involves using autologous tissue, prosthetic implants, or a combination of both with the goal of reconstructing a natural-looking breast. This process often also includes the rebuilding of the nipple and areola, known as nipple-areola complex (NAC) reconstruction, as one of the final stages.
Mastectomy is the medical term for the surgical removal of one or both breasts, partially or completely. A mastectomy is usually carried out to treat breast cancer. In some cases, women believed to be at high risk of breast cancer have the operation as a preventive measure. Alternatively, some women can choose to have a wide local excision, also known as a lumpectomy, an operation in which a small volume of breast tissue containing the tumor and a surrounding margin of healthy tissue is removed to conserve the breast. Both mastectomy and lumpectomy are referred to as "local therapies" for breast cancer, targeting the area of the tumor, as opposed to systemic therapies, such as chemotherapy, hormonal therapy, or immunotherapy.
Breast cancer is a cancer that develops from breast tissue. Signs of breast cancer may include a lump in the breast, a change in breast shape, dimpling of the skin, milk rejection, fluid coming from the nipple, a newly inverted nipple, or a red or scaly patch of skin. In those with distant spread of the disease, there may be bone pain, swollen lymph nodes, shortness of breath, or yellow skin.
The nipple is a raised region of tissue on the surface of the breast from which, in females, milk leaves the breast through the lactiferous ducts to breastfeed an infant. The milk can flow through the nipple passively or it can be ejected by smooth muscle contractions that occur along with the ductal system. Male mammals also have nipples but without the same level of function, and often surrounded by body hair.
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Nipple discharge is fluid from the nipple, with or without squeezing the breast. The discharge can be milky, clear, green, purulent, bloody, or faintly yellow. The consistency can be thick, thin, sticky, or watery.
Invasive carcinoma of no special type, invasive breast carcinoma of no special type (IBC-NST), invasive ductal carcinoma (IDC), infiltrating ductal carcinoma (IDC) or invasive ductal carcinoma, not otherwise specified (NOS) is a disease. For international audiences this article will use "invasive carcinoma NST" because it is the preferred term of the World Health Organization (WHO).
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Amastia refers to a rare clinical anomaly in which both internal breast tissue and the visible nipple are absent on one or both sides. It affects both men and women. Amastia can be either isolated or comorbid with other syndromes, such as ectodermal dysplasia, Syndactyly and lipoatrophic diabetes. This abnormality can be classified into various types, and each could result from different pathologies. Amastia differs from amazia and athelia. Amazia is the absence of one or both mammary glands but the nipples remain present, and athelia is the absence of one or both nipples, but the mammary gland remains.
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Extramammary Paget's disease (EMPD) is a rare and slow-growing malignancy which occurs within the epithelium and accounts for 6.5% of all Paget's disease. The clinical presentation of this disease is similar to the characteristics of mammary Paget's disease (MPD). However, unlike MPD, which occurs in large lactiferous ducts and then extends into the epidermis, EMPD originates in glandular regions rich in apocrine secretions outside the mammary glands. EMPD incidence is increasing by 3.2% every year, affecting hormonally-targeted tissues such as the vulva and scrotum. In women, 81.3% of EMPD cases are related to the vulva, while for men, 43.2% of the manifestations present at the scrotum.
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