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Paget Disease of the Nipple

Paget's Disease of the Nipple

Paget's disease of the breast is a form of cancer that primarily affects the skin of the nipple and sometimes the areola as well. It is a rare ailment that was first described in 1874 by Sir James Paget. Many cases of Paget's disease are associated with an underlying breast carcinoma.


Presentation

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.

Hunting
  • Kawase K, Dimaio DJ, Tucker SL, Buchholz TA, Ross MI, Feig BW, Kuerer HM, Meric-Bernstam F, Babiera G, Ames FC, Singletary SE, Hunt KK (2005) Paget’s disease of the breast: there is a role for breast-conserving therapy. Ann Surg Oncol 12(5):391–397.[doi.org]
Heart Disease
  • Diet and exercise Exercising regularly and eating a healthy,balanced diet are known to help prevent many forms of cancer, as well as other serious health conditions, such as heart disease and diabetes.[your.md]
  • Diet and lifestyle Exercising regularly and eating a healthy, balanced diet are known to help prevent many forms of cancer, as well as other serious health conditions, such as heart disease and diabetes .[nhsdirect.wales.nhs.uk]
  • Diet and lifestyle Exercising regularly and eating a healthy, balanced diet are known to help prevent many forms of cancer, as well as other serious health conditions, such as heart disease and diabetes.[nhsinform.scot]
Cutaneous Manifestation
  • J Can Res Ther 2010;6:95-6 Introduction Since the initial description in 1874, 2 Cutaneous manifestations of male breast cancer Reginster, M.A., Quatresooz, P., Piérarrarrard-Fraranchimont, C., Piérarrarrard, G.E.[cancerjournal.net]
  • Abstract Paget's disease of the breast nipple and areola complex represents a cutaneous manifestation of an underlying breast malignancy.[ncbi.nlm.nih.gov]
  • Sánchez, Cutaneous Manifestations of Internal Malignancy and Paraneoplastic Syndromes, Atlas of Dermatology in Internal Medicine, 10.1007/978-1-4614-0688-4_6, (59-76), (2011). D. Geffroy, I. Doutriaux-Dumoulins, C. Labbe-Devilliers, P. Meingan, S.[doi.org]
Papule
  • This patient has lymphomatoid papulosis, a condition in which groups of pruritic papules at different stages of development recurrently arise mainly on the trunk and limbs.[jmedicalcasereports.biomedcentral.com]
Leg Cramp
  • Raloxifene can cause side effects including flu-like symptoms, hot flushes and leg cramps. Side effects of tamoxifen can include hot flushes and sweats, changes to your periods and nausea and vomiting.[nhsdirect.wales.nhs.uk]
Suggestibility
  • The other theory suggests that skin cells of the nipple spontaneously become Paget cells.[web.archive.org]
  • Your doctor will do a physical exam and should suggest having a mammogram of both breasts done immediately.[truthorfiction.com]
  • 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]
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
  • Paget Disease of the Breast Nipple Discharge Breast discharge is the spontaneous flow of fluid from the nipple at any time other than during nursing.[medicinenet.com]
  • The symptoms include: Itching, tingling, redness, and/or a burning sensation in the affected nipple Sensitivity and pain Flaking of scales and skin thickening of the affected nipple Flattening or turning inwards (inversion) of the nipple Discharge (yellowish[news-medical.net]
  • If you have nipple discharge, a sample of the discharge might also be collected for microscopic examination. If cancer cells are detected in the samples collected, you may be referred to a breast surgeon to discuss treatment options.[web.archive.org]
  • 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]
Ulcer of Nipple
  • […] 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]
Seizure
  • If the brain or spinal cord are affected, symptoms may include pain, confusion, memory loss, headache, blurred or double vision, difficulty with speech, difficulty with movement or seizures.[sharecare.com]

Workup

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].

Abnormal Mammogram
  • In some instances, women who show signs of Paget's disease of the nipple will also have an abnormal mammogram or present a breast lump upon physical examination.[imaginis.com]
  • Radiation without Surgery The possibility of radiation treatment alone for patients with Paget's disease without a palpable mass or abnormal mammogram has been reported.[aboutcancer.com]

Treatment

  • 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]
  • However, people who have Paget disease of the breast may be eligible to enroll in clinical trials to evaluate new treatments for breast cancer in general, new ways of using existing breast cancer treatments, or strategies for preventing breast cancer[medicinenet.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]

Prognosis

  • 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]
  • If no lump is felt in the breast tissue, and your biopsy results show the cancer has not spread, the outlook (prognosis) is excellent.[cancer.org]

Etiology

  • 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]
  • 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]

Epidemiology

  • 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]
  • 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.[medicinenet.com]
Sex distribution
Age distribution

Pathophysiology

  • 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 The pathogenesis of mammary Paget disease and the origin of Paget cells were once controversial.[web.archive.org]
  • Pathophysiology The pathogenesis of Paget’s disease of the breast still remains controversial.[wikidoc.org]

Prevention

  • 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]
  • Prevention A number of factors can help reduce your risk of developing breast cancer, including Paget's disease of the nipple.[your.md]
  • 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]
  • In most cases, radiation therapy is also used to help prevent recurrence (return of the cancer).[web.archive.org]

References

Article

  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.

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Last updated: 2019-07-11 20:17