Paget's disease of the breast (PDB) is a carcinoma of the breast that involves the nipple and the areola. It is a rare disease and is usually linked to carcinoma in situ, or an invasive carcinoma in the breast, which may be evidenced by a palpable mass on examination of the breast [1]. PDB constitutes only about 1-4% of reported cases of breast cancer in females [2]. About 40% of those associated with invasive cancer are associated with ductal carcinoma in situ [3]. PDB can occur in both men and women, however, it predominantly affects the latter. The disease is often diagnosed in the 5th decade, although cases of younger and older-onset have been reported [1]. PDB often presents as unilateral disease. It can occur in accessory nipples, as well as non-breast tissue after mastectomy [4] [5]. Furthermore, it has been reported to occur in the breast tissue in the axilla [6].

PDB affects the skin and causes lesions that may resemble eczema [7]. For this reason, it may be misdiagnosed as a dermatological condition. The nipple and areola are often erythematous, itchy, flaky, and sometimes there is an erosion of the nipple. A nipple discharge is not uncommon and may range from serosanguineous to purulent. Other reported findings are ulceration, bleeding, thick plaque formation, and pain that is often burning in nature.

• Suggestibility

  The areola lesion was highly suggestive of a dermatologic malignancy such as malignant melanoma. As such, a biopsy was performed with initial findings suggestive of cutaneous melanoma. [ncbi.nlm.nih.gov]

  Your doctor will do a physical exam, and should suggest having a mammogram of both breasts done immediately. [snopes.com]

  The other theory suggests that skin cells of the nipple spontaneously become Paget cells. [web.archive.org]

• Anger

  Prolonged stress may lead to frustration, anger, hopelessness, and -- at times -- depression. The person with the illness is not the only one affected. Family members are also influenced by the health changes of a loved one. [web.archive.org]

  You may have many different emotions, including anger, resentment, guilt, anxiety and fear. These are all normal reactions and are part of the process many people go through in trying to come to terms with their condition. [macmillan.org.uk]

• Nipple Discharge

  Nipple discharge: About 50% of papillary carcinomas occur beneath the nipple, resulting in bloody nipple discharge. 7 Answers A The only way to determine if a lump is cancerous or benign is through a medical examination. [sharecare.com]

  The nipple and areola are often erythematous, itchy, flaky, and sometimes there is an erosion of the nipple. A nipple discharge is not uncommon and may range from serosanguineous to purulent. [symptoma.com]

  The most common presenting symptoms were nipple discharge and eczematous changes of the nipple/areola complex. Ninety-seven patients (93.2%) had an underlying invasive or noninvasive cancer associated with Paget's disease. [ncbi.nlm.nih.gov]

  During a biopsy, your doctor obtains a small sample of tissue from the skin of your nipple for examination under a microscope. If you have nipple discharge, a sample of the discharge might also be collected for microscopic examination. [web.archive.org]

  Other symptoms include: Redness Swelling Oozing nipple discharge Scaling Bloody nipple discharge Ulceration Inversion of the nipple Scaly, crusty and thickened plaques often form on the nipple, which then spreads to the surrounding area. [dermnetnz.org]

• Ulcer of Nipple

  […] an ulcer the nipple might flatten out or turn inwards (invert) there may be a discharge or bleeding from the nipple the area might feel itchy or sore there may be a lump in the same breast. [breast-cancer.canceraustralia.gov.au]

  which may later progress to ulceration, erosion and frank destruction, bloody nipple discharge, nipple retraction, or a palpable mass. [indianjcancer.com]

Paget's disease of the breast has four stages, stage zero describing malignancy that only involves the epidermis, and stage four being PDB that is associated with invasive ductal carcinoma. There are several modalities that are used in the diagnosis of PDB. Clinical breast examination is of particular importance as up to 50% of those with PDB will have a palpable breast lump. Subsequent diagnostic techniques involve both invasive and noninvasive strategies.

Breast tissue from the affected area should be analyzed histologically, thus necessitating a biopsy. The choice of what type of biopsy to carry out ranges between wedge, punch, shave and surface biopsy. Immunochemistry is also carried out on biopsy samples. Together, the above laboratory techniques (immunohistochemistry) form the definitive diagnostic measure for PDB [8].

Imaging studies include mammography, ultrasound and magnetic resonance imaging (MRI) [1]. These are useful in detecting nonpalpable breast masses [9]. On mammography, PDB may cause distortion of the breast architecture, calcifications, edema, and nipple changes. Negative findings on mammography do not rule out the possibility of PDB, as about 70% of cases with histological evidence of the disease also have positive findings on mammography. Therefore there is a proportion of cases of PDB without mammographic features of the disease. MRI is useful in detecting PDB that otherwise goes undetected by the former [10].

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 ). [web.archive.org]

Keywords Paget’s disease of the nipple Symtoms Treatment Prognosis This is a preview of subscription content, log in to check access. References 1. [doi.org]

You may also want to talk with your doctor about participating in a clinical trial, which helps physicians learn about new treatments and better ways to use established treatments. [cblpath.com]

Treatment of underlying tumor Excision of the nipple-areolar complex Treatment of Paget disease of the nipple involves appropriate breast cancer treatment for discovered underlying tumors and includes wide excision of the nipple-areolar complex. [merckmanuals.com]

Treatment and Prognosis Mastectomy had been considered the first line of treatment for many years because patients were more commonly found to have more than one tumor within the same breast. [news-medical.net]

Patients with nipple-areolar disease alone and no palpable mass have an excellent prognosis. [ncbi.nlm.nih.gov]

Mastectomy Radiation therapy Hormonal therapy What is the prognosis for Paget’s disease? If the biopsy shows DCIS, stage 0 cancer and no invasive cancer, the prognosis is excellent. [hopkinsmedicine.org]

ICD-10-CM Codes › C00-D49 Neoplasms › C50-C50 Malignant neoplasms of breast › Use Additional Use Additional Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10data.com]

As you have alluded to, Paget's disease of bone (osteitis deformans) is a disease of uncertain etiology, which is characterized by excessive bone resorption accompanied by abnormal new bone formation. [oncolink.org]

Etiology The disease is caused by a mutation in the gene encoding 3-beta-hydroxy-delta-5-C27 steroid oxidoreductase (HSD3B7, 16p12-p11.2). Transmission is autosomal recessive. [orpha.net]

Furthermore, the breast examination reveals no untoward findings (such as a palpable mass, axillary lymphadenopathy, or nipple discharge), making a benign etiology even more likely. [prognosisapp.com]

[…] invades the skin; the invasive carcinoma is usually large in size Paget disease with dermal invasion occurs when Paget cells cross the basement membrane of the epidermis; the invasive component is usually small in size ( Arch Pathol Lab Med 2013;137:72 ) Epidemiology [pathologyoutlines.com]

Epidemiology First Online: 19 October 2007 Abstract Background Paget’s disease of the nipple is a rare form of breast cancer characterised by the presence of intraepidermal tumour cells. [doi.org]

It is a rare type of cancer of the nipple-areola complex presenting as an eczematous lesion of the nipple and is often associated with an underlying in-situ or invasive carcinoma of the breast. [ 1 ] Epidemiology It is seen in 1-4% of cases of female [patient.info]

Summary Epidemiology Prevalence is unknown but may be around 1-9/1,000,000 for overall BAS defects, excluding cerebrotendinous xanthomatosis. [orpha.net]

According to NCI’s Surveillance, Epidemiology, and End Results program, the 5-year relative survival for all women in the United States who were diagnosed with Paget disease of the breast between 1988 and 2001 was 82.6 percent. [cancer.gov]

Pathophysiology The underlying reason for the condition is not yet established. It might be that tumor growth initiates in ducts before migrating to the external surface of the nipple. [news-medical.net]

[…] breast cancer (~10% DCIS, ~90% invasive) However, Underlying breast cancer If palpable or mammographic mass, >90% will be invasive carcinoma, and >50% may have LN+ If non-palpable, 60-80% will be DCIS Work-Up Bilateral breast examination Mammogram Biopsy Pathophysiology [en.wikibooks.org]

[…] of female breast cancer. [ 2 ] It is also seen in male breast cancer but extremely rarely. [ 3 ] Most cases of Paget's disease of the breast are associated with invasive breast cancer - although 40% are associated with ductal carcinoma in situ. [ 2 ] Pathophysiology [patient.info]

Pathophysiology[edit] Paget's disease of the breast is characterised by Paget cells. [en.wikipedia.org]

Pathophysiology The pathogenesis of mammary Paget disease and the origin of Paget cells were once controversial. [web.archive.org]

Prevention Although there is no known cause, the disease cannot be prevented. At best it could be detected at an early stage, which could guarantee proper treatment and recovery. [health24.com]

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time. Find out more about the register. [nhsinform.scot]

Prevention A number of factors can help reduce your risk of developing breast cancer, including Paget's disease of the nipple. [your.md]

Prevention Because the cause of Paget's disease of the breast is not known, prevention of this disease is not possible. [medical-dictionary.thefreedictionary.com]

They usually undergo radiation therapy after the actual procedure to prevent recurrence. A breast-conserving surgery consists in the removal of the nipple, areola and the part of the breast that is affected by cancer. [en.wikipedia.org]

1. Caliskan M, Gatti G, Sosnovskikh I, et al. Paget's disease of the breast: the experience of the European Institute of Oncology and review of the literature. Breast Cancer Res Treat. 2008;112(3):513–521.
2. Subramanian A, Birch H, McAvinchey R, Stacey-Clear A. Pagets disease of uncertain origin: case report. Int Semin Surg Oncol. 2007;4:12.
3. Piekarski J, Kubiak R, Jeziorski A. Clinically silent Paget disease of male nipple. J Exp Clin Cancer Res. 2003;22(3):495-496.
4. Giovannini M, D’Atri C, Piubello Q, Molino A. Mammary Paget's disease occurring after mastectomy. World J Surg Oncol. 2006;4:51.
5. Martin VG, Pellettiere EV, Gress D, Miller AW. Paget's disease in an adolescent arising in a supernumerary nipple. J Cutan Pathol. 1994;21(3):283-286.
6. El Khoury M, Lalonde L, David J, Issa-Chergui B, Peloquin L, Trop I. Paget's disease of the axilla arising from an underlying accessory mammary tissue. Clin Radiol. 2011;66(6):575-577.
7. Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol. 2007;21(5):581–590.
8. Kurokawa I, Furukawa S, Kishimoto M, Kakuno A, Tsubura A. Keratin Expression in Mammary Paget's Disease in situ with Intraductal Invasion. Case Rep Oncol. 2012;5(2):400-403.
9. Echevarria JJ, Lopez-Ruiz JA, Martin D, Imaz I, Martin M. Usefulness of MRI in detecting occult breast cancer associated with Paget's disease of the nipple-areolar complex. Br J Radiol. 2004;77(924):1036-1039.
10. Morrogh M, Morris EA, Liberman L, Van Zee K, Cody HS 3rd, King TA. MRI identifies otherwise occult disease in select patients with Paget disease of the nipple. J Am Coll Surg. 2008;206(2):316-321.