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Paget Disease of the Breast

Mammary Paget Disease


Presentation

  • When present, the prognosis is the poorest. About 31 (62%) of 50 patients with mammary Paget disease present with a detectable breast mass.[web.archive.org]
  • Through a collaborative review of patients treated with BCS and RT from seven institutions, 38 cases of Paget disease of the breast presenting without a palpable mass or mammographic density were identified.[ncbi.nlm.nih.gov]
  • Since, the initial presentation is limited to skin; it is the dermatologist who plays a key role in making a diagnosis, thus, facilitating proper management.[ijdvl.com]
  • The prognosis, or outlook, for people with Paget disease of the breast depends on a variety of factors, including the following: Whether or not a tumor is present in the affected breast If one or more tumors are present in the affected breast, whether[cancer.gov]
Fishing
  • Eighty percent of the cases showed strong overexpression (3 ) of HER-2 protein by IHC, and all of these cases showed more than 5-fold amplification of the HER-2 gene by FISH.[ncbi.nlm.nih.gov]
Cutaneous Manifestation
  • 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]
  • Cutaneous manifestations of breast cancer. Semin Oncol. 2016 Jun. 43 (3):331-4. [Medline]. 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.[emedicine.medscape.com]
Suggestibility
  • Paget cells and underlying ductal carcinoma cells have been shown to be positive for the oncogene HER2/neu, suggesting common genetic alterations for both the epidermal and breast tumor cells.[emedicine.medscape.com]
  • The epidermotropic theory suggests that Paget's cells are ductal carcinoma cells that have migrated from an underlying carcinoma of the breast parenchyma to the epidermis of the nipple.[ncbi.nlm.nih.gov]
Nipple Discharge
  • 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]
  • 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]
  • 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]
  • She reported the appearance of eczema of the nipple-areola complex some years before, and the presence of a palpable mass for about two months; no mention was made of nipple discharge or ulceration.[wjso.biomedcentral.com]
Bloody Nipple Discharge
  • Possible signs and symptoms of Paget's disease of the breast include: Flaky or scaly skin on your nipple Crusty, oozing or hardened skin resembling eczema, on the nipple, areola or both Redness Itching A burning sensation Straw-colored or bloody nipple[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]
Bloody Nipple Discharge
  • Possible signs and symptoms of Paget's disease of the breast include: Flaky or scaly skin on your nipple Crusty, oozing or hardened skin resembling eczema, on the nipple, areola or both Redness Itching A burning sensation Straw-colored or bloody nipple[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]
Bleeding from the Nipple

Workup

  • Proper recognition of this disorder is required to initiate an appropriate workup (eg, skin biopsy) for differentiating it from other benign inflammatory dermatosis and for detecting an underlying breast carcinoma.[web.archive.org]
Lymphocytic Infiltrate
  • infiltrate in the dermis, and positivity for EMA and Her - 2 / neu favored a diagnosis of Paget's disease.[ijdvl.com]

Treatment

  • We propose a treatment algorithm for patients presenting with Paget's disease of the breast.[ncbi.nlm.nih.gov]
  • Surgery Surgery is the main treatment for Paget’s disease.[web.archive.org]
  • The histogenesis of Paget's disease of the breast continues to be debated and is important when considering treatment options.[doi.org]

Prognosis

  • The presence of three factors for the prognosis has been suggested, whether there is a palpable mass of the disease, whether lymph nodes are positive and whether there is an underlying malignant cancer.[en.wikipedia.org]
  • Patients generally have a very good prognosis depending on the extent of the malignancy.[aocd.org]
  • The prognosis for extramammary Paget's disease secondary to underlying neoplasia is worse than when the disease has arisen primarily within the epidermis. It depends on the prognosis of the underlying carcinoma.[doi.org]

Etiology

  • […] genitalia and perineal skin may be associated with bladder, urethra, prostate cancer, or colorectal neoplasia. [3] Due to such associations, a complete investigation for an underlying carcinoma should accompany every confirmed diagnosis of EMPD. [4] Etiology[e-ijd.org]
  • The purpose of the work is to make a clinical laboratory evaluation of the role of urogenital opportunistic pathogenic flora in etiology and pathogenesis of pathological uterine cervix conditions and to identify possible preventive measures to uterine[giog.it]
  • Etiology MPD and EMPD are neoplastic processes. The causes and cells of origin are controversial. Pathophysiology MPD is largely believed to originate from underlying ductal carcinoma of the breast.[dermatologyadvisor.com]

Epidemiology

  • Epidemiology Paget's disease of the breast, a disorder of the nipple-areola complex, first described by Sir James Paget in 1874, 2 Dermatopathology of the Female Breast Margot S. Peters,Julia S. Lehman,Nneka I.[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.[cancer.gov]
  • 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

  • In triple or quadruple extra-mammary Paget’s disease, the racial and gender differences in incidence, the pathophysiology underlying simultaneous distant occurrence of three or four lesions, and their malignant potentials are the matters to be elucidated[medicaljournals.se]
  • Pathophysiology The pathogenesis of mammary Paget disease and the origin of Paget cells were once controversial.[web.archive.org]
  • Pathophysiology Paget's disease of the breast is characterised by Paget cells.[ipfs.io]
  • […] 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 MPD is largely believed to originate from underlying ductal carcinoma of the breast. Some, however, question whether Toker cells beget MPD, especially in the uncommon instances of MPD arising without underlying breast carcinoma.[dermatologyadvisor.com]

Prevention

  • 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]
  • 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]
  • Breast Cancer - Get the facts on breast cancer symptoms, signs, stages, types, research, risk factors, diagnosis, prevention information and treatment options: mastectomy, chemotherapy, radiation therapy.[web.archive.org]

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