Materiały źródłowe

• #1 Mammary Paget Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563228/

  Paget’s disease of the breast is an uncommon manifestation of underlying breast cancer in postmenopausal female patients. Underlying breast lesion is usually a ductal carcinoma in situ (DCIS) but can be invasive cancer. […] It is widely accepted that Paget disease is associated with some underlying breast malignancy, usually ductal carcinoma in situ (DCIS) or invasive ductal carcinoma. It is theorized that these malignant ductal epithelial cells migrate toward the skin through the lactiferous ducts and ductules. […] A couple of hypotheses have been proposed to explain the pathogenesis of PDB. […] Epidermotropic theory: proposes the Paget cell arises from an underlying mammary adenocarcinoma, with the neoplastic ductal epithelial cells migrating through the ductal system of the breast and reaching the epidermis of the nipple.

• #2 Paget’s disease of the breast

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3263015/

  Paget’s disease of the breast is a rare type of cancer of the nippleareola complex and that is often associated with an underlying in situ or invasive carcinoma. […] Its clinical significance, pathogenesis, and surgical treatment remain topics of clinical debate. […] Paget’s disease is almost always associated with an underlying in situ or invasive cancer or both. […] The first theory, epidermotropic theory, suggests that the Paget’s cells originate from ductal cancer cells that have migrated along the basal membrane of the nipple. […] The second theory, called in situ malignant transformation or degeneration from existing cells, regards the Paget’s cells as malignant keratinocytes appearing in situ, and therefore considers Paget’s disease of the breast to be an in situ carcinoma, independent of any underlying carcinoma. […] The ras oncogene product p21 overexpression has been demonstrated in mammary and extramammary Paget’s disease. […] Also, recently, some studies suggested that NY-BY-1 expression or loss of pRb expression may have a role to play in the pathogenesis of Paget’s disease.

• #3 Paget`s disease of the Breast. Differential diagnosis using additional IHC-panel. Literature review and own observations. – Unified Citation Journals

  https://ucjournals.com/pagets-disease-of-the-breast-differential-diagnosis-using-additional-ihc-panel-literature-review-and-own-observations/

  In 1874, Sir James Paget described a tumor that was later called Pagets disease of the breast. Currently, Pagets disease of the breast is a rare type of cancer that affects the skin of the nipple and usually the areola. Moreover, it should be noted that, as a rule, this cancer is associated with other breast carcinomas, such as: ductal carcinoma in situ or invasive cancer. […] For almost 200 years of studying the disease, dozens of different theories of pathogenesis have been proposed, refuted, and again proposed: […] In the 1889 year. Jean Darier et al gave a very detailed description of the histopathological changes that occur in Pagets mammary disease. The authors considered Pagets cells to be parasitic (sporospermia or coccidia ells), which provoked Pagets carcinoma. […] In 1902 year. Darier has safely abandoned the previously stated parasitic theory. Since then, he believed that Pagets disease arises from the epidermis and excretory ducts of the mammary gland, due to their degenerative and dystrophic changes, and Pagets cells are special epithelial elements, having undergone a specific type of cellular degeneration.

• #4 Paget’s disease of the breast pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Paget%27s_disease_of_the_breast_pathophysiology

  The pathogenesis of Pagets disease of the breast still remains controversial. […] The commonly accepted hypothesis is that most cases of Paget’s disease of the breast originate from in situ or invasive ductal carcinoma of the underlying breast tissue. This is supported by two different theories: […] According to this hypothesis malignant epithelial cells from intraductal carcinoma, extend into the overlying epidermis through mammary duct epithelium and proliferate in the epidermis causing thickening of the nipple and areolar skin. […] Normal epidermal keratinocytes produce and release the mobility factor heregulin-alpha which is chemotactic for heregulin receptors (Her-2) and coreceptors Her 3 and Her 4 which are produced by Pagets cells. This is thought to result in migration of these cells to the nipple epidermis.

• #5 :: Journal of Breast Disease

  https://www.jbd.or.kr/m/journal/view.php?number=126

  Intramammary Pagets disease is an uncommon disease in which 90% of the cases are accompanied by invasive or noninvasive ductal carcinoma. […] The pathogenesis of intramammary Pagets disease continues to be debated, with two main theories involved. The epidermotropic theory states that Pagets cells are ductal carcinoma cells that have migrated from the underlying breast parenchyma to the nipple epidermis, known as Pagetoid spread. […] In the in situ transformation theory, the Pagets cells arise as malignant cells in the nipple epidermis, independent from any other pathologic process within the breast parenchyma. […] Pagets cells do not invade through the dermal basement membrane and therefore are a form of carcinoma in situ. […] Intramammary Pagets disease with dermal invasion has been reported rarely worldwide, and its management and prognosis remain unknown.

• #6 Pathology Outlines – Paget disease

  https://www.pathologyoutlines.com/topic/breastmalignantpaget.html

  Mammary Paget disease (MPD) is a proliferation of malignant glandular epithelial cells (in situ carcinoma) in the nipple areolar epidermis (Histopathology 2020;77:181) […] 2 theories on the pathogenesis: […] Epidermotropic theory (most widely accepted): Paget cells are DCIS cells that migrate along the basement membrane of the nipple, supported by the presence of DCIS deeper in the breast identical to Paget cells in almost all cases; migration may be mediated through a motility factor heregulin α, produced by keratinocytes and exerting its effect via the HER2 receptor (J Natl Cancer Inst 2000;92:622) […] Transformation theory: Paget cells originate from malignant transformation of keratinocytes or Toker cells; it would explain rare cases (< 5%) in which cancer is not present in underlying breast (Intractable Rare Dis Res 2019;8:203) [...] When no parenchymal cancer is identified, there is a possibility that the underlying tumor has gone undetected / unsampled.

• #7 Paget’s disease of the breast – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-the-breast/symptoms-causes/syc-20351079

  Paget’s disease of the breast is a rare form of breast cancer. […] The most widely accepted theory is that the disease results from an underlying ductal breast cancer. The cancer cells from the original tumor then travel through milk ducts to the nipple and its surrounding skin. […] Another theory is that the disease can develop independently in the nipple.

• #8 Paget Disease of the Breast – NCI

  https://www.cancer.gov/types/breast/paget-breast-fact-sheet

  Paget disease of the breast is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, which is called the areola. Most people with Paget disease of the breast also have one or more tumors inside the same breast. These breast tumors are either ductal carcinoma in situ or invasive breast cancer. […] Doctors do not fully understand what causes Paget disease of the breast. The most widely accepted theory is that cancer cells from a tumor inside the breast travel through the milk ducts to the nipple and areola. This would explain why Paget disease of the breast and tumors inside the same breast are almost always found together. […] A second theory is that cells in the nipple or areola become cancerous on their own. This would explain why a few people develop Paget disease of the breast without having a tumor inside the same breast. Moreover, it may be possible for Paget disease of the breast and tumors inside the same breast to develop independently.

• #9 Understanding Paget’s Disease Accompanied by Invasive Breast Cancer – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2020/december-2020-vol-11-no-12/understanding-paget-s-disease-accompanied-by-invasive-breast-cancer

  Because these underlying malignancies tend to be multifocal and multicentric, mammography often is not sufficient, and an MRI may be necessary to evaluate the true extent of the disease. Treatment of this form of breast cancer is typically multifactorial in that clear margins versus final cosmesis must be considered. As this is generally located in the central part of the breast, it may be difficult to preserve the nipple areolar complex, and simple mastectomy with sentinel lymph node dissection is often the best approach. It has been noted in recent literature that both total mastectomy and breast-preserving surgery followed by appropriate adjuvant therapy are acceptable treatment options for carefully selected patients with Pagets disease. […] About 97% of people diagnosed with Pagets disease of the nipple also have ductal carcinoma in situ or invasive cancer somewhere else in the breast. The exact treatment plan will depend on the characteristics of the underlying breast cancer such as stage, grade, and whether the cancer is hormone receptor-positive and/or HER2-positive.

• #10 Paget’s Disease of the Breast: Symptoms, Treatment & Prognosis

  https://www.cancercenter.com/cancer-types/breast-cancer/types/rare-breast-cancer-types/pagets-disease-of-the-breast

  Pagets disease of the breast affects about 1 to 4 percent of breast cancer patients, according to the National Cancer Institute (NCI). […] In most cases, people with Pagets disease also have ductal carcinoma in situ (DCIS), which is a breast cancer contained within the milk ducts, or invasive breast cancer. However, Pagets disease is different from other breast cancers in that the cancerous cells are found in the skin of the breast, instead of within the breast. […] Surgery is the primary treatment option for Pagets disease. […] A minority of patients with Paget’s disease have cancer confined to the nipple itself, with no sign of cancer elsewhere in the breast. […] During surgery for a Pagets disease patient also has invasive breast cancer, doctors routinely check the lymph nodes to ensure there are no cancer cells there that require removal. […] Survival rates differ depending on the severity of Pagets disease. The five-year relative survival rate for women with Pagets disease in the United States between 1988 and 2001 was 82.6 percent, according to the NCI.

• #11 SciELO Brazil – Mammary and extramammary Paget’s disease Mammary and extramammary Paget’s disease

  https://www.scielo.br/j/abd/a/x8tdYkztBBpp5vg8RzZ5bCd/

  Paget’s disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). […] Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. […] The histogenesis of MPD is controversial. There are two main hypotheses that try to explain it and a third that would represent the combination of them: […] Epidermotropic theory states that Paget cells would be cells from the generally present underlying intraductal cancer that migrated through the basement membrane to the nipple. […] Furthermore, recent studies have shown that, in most cases, there is considerable similarity in immunohistochemical staining between MPD and the associated intraductal cancer.

• #12 Pagets disease of uncertain origin: case report | International Seminars in Surgical Oncology | Full Text

  https://issoonline.biomedcentral.com/articles/10.1186/1477-7800-4-12

  Pagets disease of the nipple presents as an eczematous lesion, occurs in 1 4% of all female breast carcinoma cases and is invariably associated with underlying malignancy either overt or occult. […] A prerequisite to the development of this condition is the presence of the nipple or at least underlying mammary tissue both of which were absent in this case. […] Paget’s disease of the breast was first described by Sir James Paget in 1874 who described „an eczematous change in the skin of the nipple preceeding an underlying mammary cancer”. […] It is thought that malignant epithelial cells from intraductal carcinoma, extend into the overlying epidermis through mammary duct epithelium and proliferate in the epidermis causing thickening of the nipple and areolar skin. This is supported by the observation that Paget cells often share cell surface markers with the underlying breast carcinoma (e.g CAM 5.2, CEA, c-erb 2 and EMA).

• #13 Annals of Clinical & Laboratory Science

  https://www.annclinlabsci.org/content/39/1/17.full

  These results suggest that different molecular mechanisms may be involved for MPD depending on the types of underlying lesions, and that different panels of markers should be used to predict MPD depending on its underlying lesions. […] Second, HER2 over-expression and HER2 subtype seem to differ between MPD-associated DCIS and MPD-associated IDC. […] These observations suggest that HER2 over-expression and HER2 subtype are not interchangeable, and over-expression of HER2 and its relationship with the expression of ER and PR may be important in the pathogenesis of MPD. […] Third, MPD and non-MPD nipple-involved tumors, although both have nipple involvement, are subgroups with very different expression patterns and subtype distribution, suggesting that distinct mechanisms are involved in their pathogenesis. […] In summary, the significant differences in ER expression, PR expression, and subtype distribution between MPD associated with two underlying lesions (DCIS and IDC) suggest that different panels of markers should be used to predict MPD in these two lesions.

• #14 Paget’s disease of the breast pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Paget%27s_disease_of_the_breast_pathophysiology

  The pathogenesis of Pagets disease of the breast still remains controversial. […] The commonly accepted hypothesis is that most cases of Paget’s disease of the breast originate from in situ or invasive ductal carcinoma of the underlying breast tissue. This is supported by two different theories: […] According to this hypothesis malignant epithelial cells from intraductal carcinoma, extend into the overlying epidermis through mammary duct epithelium and proliferate in the epidermis causing thickening of the nipple and areolar skin. […] Normal epidermal keratinocytes produce and release the mobility factor heregulin-alpha which is chemotactic for heregulin receptors (Her-2) and coreceptors Her 3 and Her 4 which are produced by Pagets cells. This is thought to result in migration of these cells to the nipple epidermis.

• #15 Pagets disease of uncertain origin: case report | International Seminars in Surgical Oncology | Full Text

  https://issoonline.biomedcentral.com/articles/10.1186/1477-7800-4-12

  Normal epidermal keratinocytes produce and release the mobility factor heregulin-alpha which is chemotactic for heregulin receptors (Her-2) and coreceptors Her 3 and Her 4 which are produced by Pagets cells. This is thought to result in migration of these cells to the nipple epidermis. […] Others believe that Paget’s cells are derived from clear nipple epithelium (Toker) cells and that underlying intraductal carcinoma is simply coexisting with this disease. […] In our case, following mastectomy and latissimus dorsi reconstruction, a focus of Paget’s disease was found in the scar bridging the breast and transplanted skin. Neither of these areas are apocrine rich and therefore would be unlikely candidates for extramammary Paget’s disease. In the absence of a nipple, underlying ductal epithelium or breast tissue in general it is also very hard to explain as part of mammary Paget’s disease. […] In this case, neoplastic cells from an axillary apocrine carcinoma may have migrated up the hair follicle and along the surgical scar but the exact pathogenesis still remains unclear.

• #16 Pathology Outlines – Paget disease

  https://www.pathologyoutlines.com/topic/breastmalignantpaget.html

  Mammary Paget disease (MPD) is a proliferation of malignant glandular epithelial cells (in situ carcinoma) in the nipple areolar epidermis (Histopathology 2020;77:181) […] 2 theories on the pathogenesis: […] Epidermotropic theory (most widely accepted): Paget cells are DCIS cells that migrate along the basement membrane of the nipple, supported by the presence of DCIS deeper in the breast identical to Paget cells in almost all cases; migration may be mediated through a motility factor heregulin α, produced by keratinocytes and exerting its effect via the HER2 receptor (J Natl Cancer Inst 2000;92:622) […] Transformation theory: Paget cells originate from malignant transformation of keratinocytes or Toker cells; it would explain rare cases (< 5%) in which cancer is not present in underlying breast (Intractable Rare Dis Res 2019;8:203) [...] When no parenchymal cancer is identified, there is a possibility that the underlying tumor has gone undetected / unsampled.

• #17 PAGET’S disease of nipple – Pathology Made Simple

  https://ilovepathology.com/pagets-disease-of-nipple/

  Rare manifestation of breast cancer. Occurs in 1% to 4% of cases Named after Sir James Paget, who was a British surgeon and surgical pathologist who found an association between breast cancer and scaly lesion of the nipple. […] The malignant cells from the underlying Ductal carcinoma in situ (DCIS) or an invasive carcinoma extend via lactiferous duct into the surface of nipple. The basement membrane is not breached. Once these malignant cells reach the surface of the nipple, the normal epithelial barrier is disrupted thereby allowing the extracellular fluid to seep into the epithelial surface.

• #18 Paget’s disease of the breast – Wikipedia

  https://en.wikipedia.org/wiki/Paget%27s_disease_of_the_breast

  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. […] There is some controversy as to whether these cancer cells travel through the ductal system of the breast to the nipple, or whether these cells result from in situ malignant transformation. […] 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. […] 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.

• #19 Mammary Paget Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563228/

  Transformation theory: proposes that the PDB arises from epidermal keratinocytes independent of underlying breast malignancy and actually represents an epidermal carcinoma in situ. […] The histologic hallmark of PDB is the presence of Paget cells, which are malignant, intraepithelial adenocarcinoma cells of variable sizes usually large in size, present singly or in the form of small groups within the epidermis of the nipple. […] Toker cells are benign and have been found in about 10% of normal nipples and rarely in supernumerary nipples. Like Paget cells of PDB, Toker cells contain prominent, clear vacuolated cytoplasm, and they are considered benign counterparts of Paget cells. […] The prognosis of PDB is dependent upon the initial presentation of the disease and the presence of an underlying invasive ductal carcinoma or axillary node metastases.

• #20 Paget’s disease of the breast

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3263015/

  Paget’s disease of the breast is a rare type of cancer of the nippleareola complex and that is often associated with an underlying in situ or invasive carcinoma. […] Its clinical significance, pathogenesis, and surgical treatment remain topics of clinical debate. […] Paget’s disease is almost always associated with an underlying in situ or invasive cancer or both. […] The first theory, epidermotropic theory, suggests that the Paget’s cells originate from ductal cancer cells that have migrated along the basal membrane of the nipple. […] The second theory, called in situ malignant transformation or degeneration from existing cells, regards the Paget’s cells as malignant keratinocytes appearing in situ, and therefore considers Paget’s disease of the breast to be an in situ carcinoma, independent of any underlying carcinoma. […] The ras oncogene product p21 overexpression has been demonstrated in mammary and extramammary Paget’s disease. […] Also, recently, some studies suggested that NY-BY-1 expression or loss of pRb expression may have a role to play in the pathogenesis of Paget’s disease.

• #21 Paget’s disease of the breast pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Paget%27s_disease_of_the_breast_pathophysiology

  According to this hypothesis Paget cells are keratinocytes that have undergone malignant transformation. […] Thus it is speculated that Paget’s disease is an in situ carcinoma and is independent from any underlying parenchymal carcinoma. […] It is assumed that the underlying intraductal carcinoma coexisting with this disease is unrelated to the overlying eczematous change. […] This theory is further supported by: […] Cases suggesting the Paget cells may have originated intraepidermally, by the presence of microvilli and desmosomal attachments between Paget cells and keratinocytes. […] Cases in which there is genetic variation between Paget cells and cells of the underlying carcinoma.

• #22 Pathology Outlines – Paget disease

  https://www.pathologyoutlines.com/topic/breastmalignantpaget.html

  Mammary Paget disease (MPD) is a proliferation of malignant glandular epithelial cells (in situ carcinoma) in the nipple areolar epidermis (Histopathology 2020;77:181) […] 2 theories on the pathogenesis: […] Epidermotropic theory (most widely accepted): Paget cells are DCIS cells that migrate along the basement membrane of the nipple, supported by the presence of DCIS deeper in the breast identical to Paget cells in almost all cases; migration may be mediated through a motility factor heregulin α, produced by keratinocytes and exerting its effect via the HER2 receptor (J Natl Cancer Inst 2000;92:622) […] Transformation theory: Paget cells originate from malignant transformation of keratinocytes or Toker cells; it would explain rare cases (< 5%) in which cancer is not present in underlying breast (Intractable Rare Dis Res 2019;8:203) [...] When no parenchymal cancer is identified, there is a possibility that the underlying tumor has gone undetected / unsampled.

• #23 Paget’s disease of the breast: Causes, symptoms, treatment, and more

  https://www.medicalnewstoday.com/articles/192362

  Pagets disease of the breast typically occurs as a manifestation of a type of precancerous lesion known as ductal carcinoma in situ (DCIS) or an underlying invasive cancer. […] Pagets disease of the breast causes skin symptoms on the breasts or nipples because of underlying breast cancer. In 80% to 90% of people with Pagets disease of the breast, the underlying cancer is either ductal carcinoma in situ (DCIS) or invasive breast cancer. […] Doctors do not know the exact cause of Pagets disease of the breast. One theory is that it may be a result of a person already having breast cancer, and that Pagets disease occurs when some cancer cells migrate through the milk ducts. […] Another possible explanation is that the skin cells of the nipple spontaneously transform into cancer cells. Some people with Pagets disease of the breast have no underlying breast cancer or the tumor has no links to the disease in the nipple.

• #24 Paget’s disease of the breast pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Paget%27s_disease_of_the_breast_pathophysiology

  According to this hypothesis Paget cells are keratinocytes that have undergone malignant transformation. […] Thus it is speculated that Paget’s disease is an in situ carcinoma and is independent from any underlying parenchymal carcinoma. […] It is assumed that the underlying intraductal carcinoma coexisting with this disease is unrelated to the overlying eczematous change. […] This theory is further supported by: […] Cases suggesting the Paget cells may have originated intraepidermally, by the presence of microvilli and desmosomal attachments between Paget cells and keratinocytes. […] Cases in which there is genetic variation between Paget cells and cells of the underlying carcinoma.

• #25 Paget’s disease of the breast pathophysiology – wikidoc

  https://www.wikidoc.org/index.php/Paget%27s_disease_of_the_breast_pathophysiology

  According to this hypothesis Paget cells are keratinocytes that have undergone malignant transformation. […] Thus it is speculated that Paget’s disease is an in situ carcinoma and is independent from any underlying parenchymal carcinoma. […] It is assumed that the underlying intraductal carcinoma coexisting with this disease is unrelated to the overlying eczematous change. […] This theory is further supported by: […] Cases suggesting the Paget cells may have originated intraepidermally, by the presence of microvilli and desmosomal attachments between Paget cells and keratinocytes. […] Cases in which there is genetic variation between Paget cells and cells of the underlying carcinoma.

• #26 Mammary Paget Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563228/

  Paget’s disease of the breast is an uncommon manifestation of underlying breast cancer in postmenopausal female patients. Underlying breast lesion is usually a ductal carcinoma in situ (DCIS) but can be invasive cancer. […] It is widely accepted that Paget disease is associated with some underlying breast malignancy, usually ductal carcinoma in situ (DCIS) or invasive ductal carcinoma. It is theorized that these malignant ductal epithelial cells migrate toward the skin through the lactiferous ducts and ductules. […] A couple of hypotheses have been proposed to explain the pathogenesis of PDB. […] Epidermotropic theory: proposes the Paget cell arises from an underlying mammary adenocarcinoma, with the neoplastic ductal epithelial cells migrating through the ductal system of the breast and reaching the epidermis of the nipple.

• #27 Paget Disease of Breast

  https://www.webpathology.com/images/breast/breast-carcinomas/paget-disease-of-breast

  Paget disease (PD) of the nipple and areola is a rare form of intraepidermal adenocarcinoma that is almost always associated with in-situ or invasive breast cancer. PD arises by intraepidermal migration of ductal carcinoma cells through the lactiferous ducts to the surface epithelium of the nipple and areola. The rare cases not associated with breast carcinoma are thought to arise from the epidermal Toker cells. Paget cells infiltrate the entire thickness of epidermis singly or in nests. They have voluminous clear or pale eosinophilic cytoplasm, vesicular nuclei, prominent nucleoli, and increased mitotic activity. Glandular and acinar structures and signet ring cells with intracytoplasmic mucin may be present.

• #28 Paget Disease of Breast

  https://www.webpathology.com/images/breast/breast-carcinomas/paget-disease-of-breast

  Paget disease (PD) of the nipple and areola is a rare form of intraepidermal adenocarcinoma that is almost always associated with in-situ or invasive breast cancer. PD arises by intraepidermal migration of ductal carcinoma cells through the lactiferous ducts to the surface epithelium of the nipple and areola. The rare cases not associated with breast carcinoma are thought to arise from the epidermal Toker cells. Paget cells infiltrate the entire thickness of epidermis singly or in nests. They have voluminous clear or pale eosinophilic cytoplasm, vesicular nuclei, prominent nucleoli, and increased mitotic activity. Glandular and acinar structures and signet ring cells with intracytoplasmic mucin may be present.

• #29 Paget Disease of Breast

  https://www.webpathology.com/images/breast/breast-carcinomas/paget-disease-of-breast

  Paget disease (PD) of the nipple and areola is a rare form of intraepidermal adenocarcinoma that is almost always associated with in-situ or invasive breast cancer. PD arises by intraepidermal migration of ductal carcinoma cells through the lactiferous ducts to the surface epithelium of the nipple and areola. The rare cases not associated with breast carcinoma are thought to arise from the epidermal Toker cells. Paget cells infiltrate the entire thickness of epidermis singly or in nests. They have voluminous clear or pale eosinophilic cytoplasm, vesicular nuclei, prominent nucleoli, and increased mitotic activity. Glandular and acinar structures and signet ring cells with intracytoplasmic mucin may be present.

• #30 Paget`s disease of the Breast. Differential diagnosis using additional IHC-panel. Literature review and own observations. – Unified Citation Journals

  https://ucjournals.com/pagets-disease-of-the-breast-differential-diagnosis-using-additional-ihc-panel-literature-review-and-own-observations/

  Pagets mammary disease is characterized by scaly, erythematous, thickened, crusty plaques located on the nipple, with a tendency to spread to surrounding areas. […] Hyperkeratosis, parakeratosis and acanthosis may be detected in the epidermis. Large, rounded or elliptoid, malignant tumor cells with varying degrees of atypia may be found, in all layers of the epidermis and also in the deeper layers of the skin. […] Paget`s cells often compress the basal keratinocytes that lie between Pagets cells and the papillary dermis. Paget cells are located singly or in the nests. […] Pagets cells almost always express molecules such as low molecular weight keratins, EMA, c-erb B-2, and polyclonal pCEA. Increased expression of HER-2 family proteins, and variable expression of ER, PR receptors are also often detected. […] A brief summary of the main characteristics of Pagets breast disease is given below.

• #31 Mammary Paget Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563228/

  Transformation theory: proposes that the PDB arises from epidermal keratinocytes independent of underlying breast malignancy and actually represents an epidermal carcinoma in situ. […] The histologic hallmark of PDB is the presence of Paget cells, which are malignant, intraepithelial adenocarcinoma cells of variable sizes usually large in size, present singly or in the form of small groups within the epidermis of the nipple. […] Toker cells are benign and have been found in about 10% of normal nipples and rarely in supernumerary nipples. Like Paget cells of PDB, Toker cells contain prominent, clear vacuolated cytoplasm, and they are considered benign counterparts of Paget cells. […] The prognosis of PDB is dependent upon the initial presentation of the disease and the presence of an underlying invasive ductal carcinoma or axillary node metastases.

• #32 Annals of Clinical & Laboratory Science

  https://www.annclinlabsci.org/content/39/1/17.full

  Mammary Pagets disease (MPD) is a rare manifestation of breast carcinoma involving the nipple. […] MPD usually occurs in conjunction with an underlying in situ ductal (DCIS) or invasive ductal carcinoma (IDC) of the breast. […] an epidermotropic spread from the underlying carcinoma is the mechanism currently accepted by many. […] It is believed that such MPD arises from so-called Toker cells present within the epidermis of the nipple/areolar region. […] Recent reports suggest that Toker cells may be the true precursors for MPD, while the underlying tumors are coincidental neoplastic lesions. […] The present study provides three unique findings that may help elucidate MPD and its underlying lesions. First, different receptor expression patterns and molecular subtype distributions were observed in two different types of MPD-associated lesions DCIS and IDC.

• #33 Paget’s Disease | Oncohema Key

  https://oncohemakey.com/pagets-disease/

  Because not all Pagets disease is associated with an underlying carcinoma, another theory, the intraepidermal transformation theory (in situ transformation theory) proposes that the Paget cells arise either in the terminal portion of the lactiferous duct at its junction with the epidermis or from multipotential cells in the epidermal basal layer. This in situ transformation theory is thought to occur in pre-existing benign intraepidermal clear cells of the nipple areolar complex, or Toker cells, which are thought to have migrated from nonneoplastic ducts. Support for this theory is found in the rare cases of Pagets disease without an underlying breast carcinoma or cases in which the Pagets disease and the underlying carcinoma appear to be separate tumors. Other studies have identified desmosomal attachments between the Paget cells and adjacent keratinocytes supporting the in situ development of the Paget cell.

• #34 Mammary Paget’s Disease: An Update

  https://www.mdpi.com/2072-6694/14/10/2422

  Mammary Paget’s Disease is a non-invasive cutaneous malignancy of the breast involving the nipple-areolar complex that is commonly mistaken for benign breast conditions, leading to delay in diagnosis. […] This review article discusses Paget’s disease etiology, clinical presentation, differential diagnosis, diagnostic work-up, natural history and prognosis. […] Two main theories have been proposed for how MPD arises: the epidermotropic theory and the intraepidermal origin theory. The epidermotropic theory states that Paget cells originate from underlying intraductal carcinoma cells that migrated along the basement membrane into the nipple. This theory is supported by the high incidence of MPD with underlying ductal carcinoma in situ (DCIS). […] The intraepidermal origin theory states that Paget cells result from malignant transformation of pluripotent keratinocyte stem cells or cells of apocrine gland ducts in the absence of underlying carcinoma, which also provides an explanation for cases of MPD where the nipple is spared. […] A hybrid theory proposes that Paget cells can originate either epidermotrophically or intraepidermally, depending on the circumstances and local conditions.

• #35 SciELO Brazil – Mammary and extramammary Paget’s disease Mammary and extramammary Paget’s disease

  https://www.scielo.br/j/abd/a/x8tdYkztBBpp5vg8RzZ5bCd/

  The second theory, the degeneration of preexisting cells, considers that Paget cells are keratinocytes that have undergone malignant transformation and that the disease is an in situ carcinoma regardless of the underlying intraductal carcinoma. […] A third theory attempts to combine the two previous ones, suggesting that Paget cells can originate in both ways already mentioned, depending on local circumstances. […] Recent studies have shown that the expression of NY-BR-1, a breast differentiation antigen, may have a role in MPD’s pathogenesis.

• #36 Paget`s disease of the Breast. Differential diagnosis using additional IHC-panel. Literature review and own observations. – Unified Citation Journals

  https://ucjournals.com/pagets-disease-of-the-breast-differential-diagnosis-using-additional-ihc-panel-literature-review-and-own-observations/

  It is generally accepted that heregulin molecules are the cause for this chemotaxis, and mainly heregulin-alpha, which express normal cells of the epidermis and which interact with heterodimers of the HER group receptors. […] It is assumed that Pagets cells may originate from glandular stem cells or Toker epidermal cells. […] S.F. Kuan et al in 2001 reported the immunohistochemical expression of apomucins such as MUC1, MUC2, and MUC5AC in Paget cells and concluded that both Pagets cells and the underlying ductal carcinoma express the same phenotypic apomucins that are also expressed by Toker cells. […] The concept of Tumor colision was introduced between Pagets breast carcinoma and the underlying ductal carcinoma. However, this concept is based on only a few observations, so further research is needed to confirm or refute the theory of tumor collision in the context of oncogenesis of Pagets breast carcinoma.

• #37 Paget’s disease of the breast

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3263015/

  Paget’s disease of the breast is a rare type of cancer of the nippleareola complex and that is often associated with an underlying in situ or invasive carcinoma. […] Its clinical significance, pathogenesis, and surgical treatment remain topics of clinical debate. […] Paget’s disease is almost always associated with an underlying in situ or invasive cancer or both. […] The first theory, epidermotropic theory, suggests that the Paget’s cells originate from ductal cancer cells that have migrated along the basal membrane of the nipple. […] The second theory, called in situ malignant transformation or degeneration from existing cells, regards the Paget’s cells as malignant keratinocytes appearing in situ, and therefore considers Paget’s disease of the breast to be an in situ carcinoma, independent of any underlying carcinoma. […] The ras oncogene product p21 overexpression has been demonstrated in mammary and extramammary Paget’s disease. […] Also, recently, some studies suggested that NY-BY-1 expression or loss of pRb expression may have a role to play in the pathogenesis of Paget’s disease.

• #38 SciELO Brazil – Mammary and extramammary Paget’s disease Mammary and extramammary Paget’s disease

  https://www.scielo.br/j/abd/a/x8tdYkztBBpp5vg8RzZ5bCd/

  The second theory, the degeneration of preexisting cells, considers that Paget cells are keratinocytes that have undergone malignant transformation and that the disease is an in situ carcinoma regardless of the underlying intraductal carcinoma. […] A third theory attempts to combine the two previous ones, suggesting that Paget cells can originate in both ways already mentioned, depending on local circumstances. […] Recent studies have shown that the expression of NY-BR-1, a breast differentiation antigen, may have a role in MPD’s pathogenesis.

• #39 Paget’s disease of the breast

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3263015/

  Paget’s disease of the breast is a rare type of cancer of the nippleareola complex and that is often associated with an underlying in situ or invasive carcinoma. […] Its clinical significance, pathogenesis, and surgical treatment remain topics of clinical debate. […] Paget’s disease is almost always associated with an underlying in situ or invasive cancer or both. […] The first theory, epidermotropic theory, suggests that the Paget’s cells originate from ductal cancer cells that have migrated along the basal membrane of the nipple. […] The second theory, called in situ malignant transformation or degeneration from existing cells, regards the Paget’s cells as malignant keratinocytes appearing in situ, and therefore considers Paget’s disease of the breast to be an in situ carcinoma, independent of any underlying carcinoma. […] The ras oncogene product p21 overexpression has been demonstrated in mammary and extramammary Paget’s disease. […] Also, recently, some studies suggested that NY-BY-1 expression or loss of pRb expression may have a role to play in the pathogenesis of Paget’s disease.

• #40 Paget’s Disease of the Breast | CancerIndex

  http://www.cancerindex.org/clink3pb.htm

  Pagets disease is a type of breast cancer that occurs in the ducts adjacent to the nipple and areola and spreads to the skin of the nipple and the areola. […] Pagets disease is usually associated with ductal carcinoma in situ (DCIS), limited to the nipple and areola area of the breast. […] Paget’s disease (PD) is an uncommon intraepithelial adenocarcinoma with unknown pathogenesis. […] Our study demonstrated a practical histologic approach to differentiate between intraepidermal pagetoid neoplasms. […] In our study we found a significantly high COX-2 expression in Paget’s disease of the breast. We suggest that COX-2 expression and inflammatory processes may play a role in pathogenesis of the Paget’s disease of the breast.

• #41 Paget’s disease of the breast – VALINTERMED treatment in Valencia

  https://valintermed.com/en/medlibrary/disease-of-the-mammary-gland/

  Paget’s disease of the breast (PDMB) is a rare disorder characterized by erythema, eczema-like changes of the skin around the nipple, which may be accompanied by itching, redness, swelling and discharge. […] PDMB is usually associated with invasive breast cancer, which requires special attention and diagnostics for an accurate assessment of the patient’s condition. […] One of the significant contributions to the study of PPD was made in the 1980s, when it was established that skin changes can be associated with extravasation of malignant cells from the internal activity of the mammary gland. […] In recent years, studies have identified several genes associated with the development of Paget’s disease. The most significant are the BRCA1 and BRCA2 genes, which are responsible for DNA repair and regulate the cell cycle. Mutations in these genes significantly increase the risk of developing both breast cancer and BPC. In addition, the TP53 gene, which is responsible for the control of apoptosis and cell division, also plays an important role in the pathogenesis of this disease. […] Treatment of Paget’s disease of the breast requires a multidisciplinary approach. The overall strategy is a comprehensive assessment of the patient’s condition, including surgical removal of the tumor, radiation therapy, and pharmacological therapy.

• #42 Paget’s disease of the breast – VALINTERMED treatment in Valencia

  https://valintermed.com/en/medlibrary/disease-of-the-mammary-gland/

  Paget’s disease of the breast (PDMB) is a rare disorder characterized by erythema, eczema-like changes of the skin around the nipple, which may be accompanied by itching, redness, swelling and discharge. […] PDMB is usually associated with invasive breast cancer, which requires special attention and diagnostics for an accurate assessment of the patient’s condition. […] One of the significant contributions to the study of PPD was made in the 1980s, when it was established that skin changes can be associated with extravasation of malignant cells from the internal activity of the mammary gland. […] In recent years, studies have identified several genes associated with the development of Paget’s disease. The most significant are the BRCA1 and BRCA2 genes, which are responsible for DNA repair and regulate the cell cycle. Mutations in these genes significantly increase the risk of developing both breast cancer and BPC. In addition, the TP53 gene, which is responsible for the control of apoptosis and cell division, also plays an important role in the pathogenesis of this disease. […] Treatment of Paget’s disease of the breast requires a multidisciplinary approach. The overall strategy is a comprehensive assessment of the patient’s condition, including surgical removal of the tumor, radiation therapy, and pharmacological therapy.

• #43 Annals of Clinical & Laboratory Science

  https://www.annclinlabsci.org/content/39/1/17.full

  These results suggest that different molecular mechanisms may be involved for MPD depending on the types of underlying lesions, and that different panels of markers should be used to predict MPD depending on its underlying lesions. […] Second, HER2 over-expression and HER2 subtype seem to differ between MPD-associated DCIS and MPD-associated IDC. […] These observations suggest that HER2 over-expression and HER2 subtype are not interchangeable, and over-expression of HER2 and its relationship with the expression of ER and PR may be important in the pathogenesis of MPD. […] Third, MPD and non-MPD nipple-involved tumors, although both have nipple involvement, are subgroups with very different expression patterns and subtype distribution, suggesting that distinct mechanisms are involved in their pathogenesis. […] In summary, the significant differences in ER expression, PR expression, and subtype distribution between MPD associated with two underlying lesions (DCIS and IDC) suggest that different panels of markers should be used to predict MPD in these two lesions.

• #44 Annals of Clinical & Laboratory Science

  https://www.annclinlabsci.org/content/39/1/17.full

  These results suggest that different molecular mechanisms may be involved for MPD depending on the types of underlying lesions, and that different panels of markers should be used to predict MPD depending on its underlying lesions. […] Second, HER2 over-expression and HER2 subtype seem to differ between MPD-associated DCIS and MPD-associated IDC. […] These observations suggest that HER2 over-expression and HER2 subtype are not interchangeable, and over-expression of HER2 and its relationship with the expression of ER and PR may be important in the pathogenesis of MPD. […] Third, MPD and non-MPD nipple-involved tumors, although both have nipple involvement, are subgroups with very different expression patterns and subtype distribution, suggesting that distinct mechanisms are involved in their pathogenesis. […] In summary, the significant differences in ER expression, PR expression, and subtype distribution between MPD associated with two underlying lesions (DCIS and IDC) suggest that different panels of markers should be used to predict MPD in these two lesions.

• #45 Annals of Clinical & Laboratory Science

  https://www.annclinlabsci.org/content/39/1/17.full

  These results suggest that different molecular mechanisms may be involved for MPD depending on the types of underlying lesions, and that different panels of markers should be used to predict MPD depending on its underlying lesions. […] Second, HER2 over-expression and HER2 subtype seem to differ between MPD-associated DCIS and MPD-associated IDC. […] These observations suggest that HER2 over-expression and HER2 subtype are not interchangeable, and over-expression of HER2 and its relationship with the expression of ER and PR may be important in the pathogenesis of MPD. […] Third, MPD and non-MPD nipple-involved tumors, although both have nipple involvement, are subgroups with very different expression patterns and subtype distribution, suggesting that distinct mechanisms are involved in their pathogenesis. […] In summary, the significant differences in ER expression, PR expression, and subtype distribution between MPD associated with two underlying lesions (DCIS and IDC) suggest that different panels of markers should be used to predict MPD in these two lesions.

• #46 Annals of Clinical & Laboratory Science

  https://www.annclinlabsci.org/content/39/1/17.full

  These results suggest that different molecular mechanisms may be involved for MPD depending on the types of underlying lesions, and that different panels of markers should be used to predict MPD depending on its underlying lesions. […] Second, HER2 over-expression and HER2 subtype seem to differ between MPD-associated DCIS and MPD-associated IDC. […] These observations suggest that HER2 over-expression and HER2 subtype are not interchangeable, and over-expression of HER2 and its relationship with the expression of ER and PR may be important in the pathogenesis of MPD. […] Third, MPD and non-MPD nipple-involved tumors, although both have nipple involvement, are subgroups with very different expression patterns and subtype distribution, suggesting that distinct mechanisms are involved in their pathogenesis. […] In summary, the significant differences in ER expression, PR expression, and subtype distribution between MPD associated with two underlying lesions (DCIS and IDC) suggest that different panels of markers should be used to predict MPD in these two lesions.

• #47 Invasive Mammary Paget Disease with no Underlying Breast Carcinoma

  https://www.oatext.com/invasive-mammary-paget-disease-with-no-underlying-breast-carcinoma.php

  Mammary Paget disease (MPD) usually presents as a neoplasm confined to the epithelium with underlying in situ or infiltrating breast carcinoma. […] Invasive Mammary Paget Disease with no underlying breast carcinoma (InvMPD) is a rare subtype of MPD that manifests as dermatosis on the nipples skin. […] Less frequently, MPD can rarely develop dermal infiltration without underlying carcinoma (invMPD). […] However, in recent years, clinical variants of the disease have been reported to invade the dermis or lack an association with ductal carcinoma in-situ (DCIS) or invasive carcinoma. Thus, invasive mammary Paget disease with no underlying breast carcinoma is the rarest manifestation of the disease with scarce reported cases. […] More than one mechanism by which MPD can arise has been postulated: the majority representing contiguous epidermotropic transport of preexisting DCIS or invasive ductal carcinoma (IDC) via the large lactiferous ducts, and cases with no underlying DCIS or IDC probably arising from a pluripotent cell or epidermal keratinocyte undergoing metaplasia and malignant transformation within the epidermis, and subsequently invading through the basement membrane into the superficial dermis. […] InvMPD is the rarest manifestation of MPD that should be considered in cases with no in-situ or invasive carcinoma after extensive imaging and histological sampling is performed.

• #48 Invasive Mammary Paget Disease with no Underlying Breast Carcinoma

  https://www.oatext.com/invasive-mammary-paget-disease-with-no-underlying-breast-carcinoma.php

  Mammary Paget disease (MPD) usually presents as a neoplasm confined to the epithelium with underlying in situ or infiltrating breast carcinoma. […] Invasive Mammary Paget Disease with no underlying breast carcinoma (InvMPD) is a rare subtype of MPD that manifests as dermatosis on the nipples skin. […] Less frequently, MPD can rarely develop dermal infiltration without underlying carcinoma (invMPD). […] However, in recent years, clinical variants of the disease have been reported to invade the dermis or lack an association with ductal carcinoma in-situ (DCIS) or invasive carcinoma. Thus, invasive mammary Paget disease with no underlying breast carcinoma is the rarest manifestation of the disease with scarce reported cases. […] More than one mechanism by which MPD can arise has been postulated: the majority representing contiguous epidermotropic transport of preexisting DCIS or invasive ductal carcinoma (IDC) via the large lactiferous ducts, and cases with no underlying DCIS or IDC probably arising from a pluripotent cell or epidermal keratinocyte undergoing metaplasia and malignant transformation within the epidermis, and subsequently invading through the basement membrane into the superficial dermis. […] InvMPD is the rarest manifestation of MPD that should be considered in cases with no in-situ or invasive carcinoma after extensive imaging and histological sampling is performed.

• #49 Invasive Mammary Paget Disease with no Underlying Breast Carcinoma

  https://www.oatext.com/invasive-mammary-paget-disease-with-no-underlying-breast-carcinoma.php

  Mammary Paget disease (MPD) usually presents as a neoplasm confined to the epithelium with underlying in situ or infiltrating breast carcinoma. […] Invasive Mammary Paget Disease with no underlying breast carcinoma (InvMPD) is a rare subtype of MPD that manifests as dermatosis on the nipples skin. […] Less frequently, MPD can rarely develop dermal infiltration without underlying carcinoma (invMPD). […] However, in recent years, clinical variants of the disease have been reported to invade the dermis or lack an association with ductal carcinoma in-situ (DCIS) or invasive carcinoma. Thus, invasive mammary Paget disease with no underlying breast carcinoma is the rarest manifestation of the disease with scarce reported cases. […] More than one mechanism by which MPD can arise has been postulated: the majority representing contiguous epidermotropic transport of preexisting DCIS or invasive ductal carcinoma (IDC) via the large lactiferous ducts, and cases with no underlying DCIS or IDC probably arising from a pluripotent cell or epidermal keratinocyte undergoing metaplasia and malignant transformation within the epidermis, and subsequently invading through the basement membrane into the superficial dermis. […] InvMPD is the rarest manifestation of MPD that should be considered in cases with no in-situ or invasive carcinoma after extensive imaging and histological sampling is performed.

• #50 Invasive Mammary Paget Disease with no Underlying Breast Carcinoma

  https://www.oatext.com/invasive-mammary-paget-disease-with-no-underlying-breast-carcinoma.php

  Mammary Paget disease (MPD) usually presents as a neoplasm confined to the epithelium with underlying in situ or infiltrating breast carcinoma. […] Invasive Mammary Paget Disease with no underlying breast carcinoma (InvMPD) is a rare subtype of MPD that manifests as dermatosis on the nipples skin. […] Less frequently, MPD can rarely develop dermal infiltration without underlying carcinoma (invMPD). […] However, in recent years, clinical variants of the disease have been reported to invade the dermis or lack an association with ductal carcinoma in-situ (DCIS) or invasive carcinoma. Thus, invasive mammary Paget disease with no underlying breast carcinoma is the rarest manifestation of the disease with scarce reported cases. […] More than one mechanism by which MPD can arise has been postulated: the majority representing contiguous epidermotropic transport of preexisting DCIS or invasive ductal carcinoma (IDC) via the large lactiferous ducts, and cases with no underlying DCIS or IDC probably arising from a pluripotent cell or epidermal keratinocyte undergoing metaplasia and malignant transformation within the epidermis, and subsequently invading through the basement membrane into the superficial dermis. […] InvMPD is the rarest manifestation of MPD that should be considered in cases with no in-situ or invasive carcinoma after extensive imaging and histological sampling is performed.

• #51 Pathological and radiological assessments of Paget’s disease – Nakai – Annals of Breast Surgery

  https://abs.amegroups.org/article/view/5150/html

  Pagets disease (PD) of the nipple was first reported and described as an eruption on the nipple and areola by Sir James Paget in 1874. Several studies have since provided evidence supporting the concept that PD is derived from an underlying adenocarcinoma. It is indeed an in situ disease with generally very favourable outcomes, and thus should be treated as an early breast cancer, not requiring systemic therapies after surgery. […] Because PD is located in the centre of the breast, total mastectomy has been the first choice for treating PD. If a patient requests central lumpectomy (CL), deformity of the breast, caused by the surgery and subsequent radiation therapy, is inevitable. The presence of a co-existing intra-breast lesion is another challenging management issue. It has also been reported that invasive or in situ ductal carcinoma co-exists with PD in 82% to 94% of cases. These intra-breast lesions might exist multifocally and multicentrally, such that precise preoperative assessment by imaging is also important for selecting the surgical procedure.

• #52 Understanding Paget’s Disease Accompanied by Invasive Breast Cancer – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2020/december-2020-vol-11-no-12/understanding-paget-s-disease-accompanied-by-invasive-breast-cancer

  Pagets disease of the breast is named after the 19th-century British doctor Sir James Paget, who, in 1874, noted a distinct connection relationship between cancer of the breast and the nipple. It is considered one of the rare forms of breast cancer and is often misdiagnosed when first noted. Typically noted in older, postmenopausal women, it comprises approximately 5% of all breast cancer cases diagnosed. The eczematous presentation of Pagets disease of the nipple is the most misleading feature of this disease. Such patients are usually mistakenly treated by the primary care physician for eczema. The initial skin changes of Pagets disease of the breast may appear relatively benign, and many individuals may overlook such symptoms, mistakenly attributing them to an inflammatory skin condition or infection. As a result, diagnosis may be delayed, often up to 6 months or more. Most individuals with the condition eventually seek medical attention, but generally only after other topical modalities have been used. This may lead to progression of disease, eventually resulting into an associated lump with enlarged axillary lymph nodes.

• #53 Paget’s Disease of Breast: One-year Experience of a Superspecialty Cancer Hospital

  https://www.jmgumst.com/abstractArticleContentBrowse/JMGUMST/26936/JPJ/fullText

  An eczematous lesion on the nipple is a hallmark of presentation. […] Risk factors for Pagets disease of the breast are similar factors that affect the risk of developing any other type of breast carcinoma such as age, personal history or family history of breast cancer, inherited gene mutation, women with dense breast tissue, radiation exposure, excess weight, hormone replacement (estrogen) after menopause.6 […] Biopsy, either of the involved nipple skin and/or a palpable lump is the definitive diagnostic method of Pagets disease.7 Biopsy can identify underlying cancer in approximately 90% of cases of Pagets disease.7 […] On immunohistochemical examination tumor cells in Pagets disease show positivity for – CK7, CAM 5.2, HER2, CEA, ER, PR, and are negative for – HMB45, Melan A, CK5 / 6, CK20.8 […] Pagets disease is an intraepidermal adenocarcinoma that is difficult to diagnose clinically as it mimics inflammatory or infectious diseases.

• #54 Invasive Paget’s Disease of the Breast: A case Report

  http://www.jbd.or.kr/journal/view.php?doi=10.14449/jbd.2020.8.2.143

  The morphological and clinical features of intra- and extramammary Pagets disease are similar; however, they differ in invasiveness. […] When biopsy of the nipple-areolar complex is positive for Pagets disease, breast MRI is recommended to define the extent of disease and identify additional diseases. […] Traditionally, management of intramammary Pagets disease has been considered to be total mastectomy with ipsilateral axillary dissection. In recent years; however, breast conserving surgery is recommended. […] There is no clear information on the clinical outcome or post-operative management of invasive intramammary Pagets disease due to its rare incidence. […] Due to its scarcity, there is still little information regarding its management and prognosis. […] Further studies are necessary to determine the usefulness of sentinel lymph node biopsy and establish management protocols for invasive intramammary Pagets disease.

• #55 Paget’s Disease of Breast: One-year Experience of a Superspecialty Cancer Hospital

  https://www.jmgumst.com/abstractArticleContentBrowse/JMGUMST/26936/JPJ/fullText

  An eczematous lesion on the nipple is a hallmark of presentation. […] Risk factors for Pagets disease of the breast are similar factors that affect the risk of developing any other type of breast carcinoma such as age, personal history or family history of breast cancer, inherited gene mutation, women with dense breast tissue, radiation exposure, excess weight, hormone replacement (estrogen) after menopause.6 […] Biopsy, either of the involved nipple skin and/or a palpable lump is the definitive diagnostic method of Pagets disease.7 Biopsy can identify underlying cancer in approximately 90% of cases of Pagets disease.7 […] On immunohistochemical examination tumor cells in Pagets disease show positivity for – CK7, CAM 5.2, HER2, CEA, ER, PR, and are negative for – HMB45, Melan A, CK5 / 6, CK20.8 […] Pagets disease is an intraepidermal adenocarcinoma that is difficult to diagnose clinically as it mimics inflammatory or infectious diseases.

• #56 Paget disease of the breast | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/breast/what-is-breast-cancer/cancerous-tumours/paget-disease-of-the-breast

  Paget disease of the breast is a rare type of breast cancer. It develops as a rash or other skin changes on the nipple, usually on only one breast. […] Most women with Paget disease also have invasive ductal carcinoma or ductal carcinoma in situ (DCIS). The cancer can then spread to the dark-coloured skin around the nipple (called the areola). […] If you have Paget disease of the breast, your healthcare team will create a treatment plan just for you. Surgery is usually the main treatment. You may be offered other treatments based on whether or not you have invasive ductal carcinoma or DCIS and if the cancer has spread to lymph nodes. These treatments may include radiation therapy, hormonal therapy, chemotherapy or targeted therapy.

• #57 Paget’s Disease of Breast: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pagets-disease-of-breast

  The surgical treatment of Paget’s disease is controversial. Mastectomy with or without axillary lymph node dissection has been regarded as the standard therapy for Paget’s disease, even in the absence of other clinical signs of malignancy. […] Delay in presentation or diagnosis is common, with diagnosis made 12 months after onset in women and 8-9 months in men. Prognosis is therefore not as good as for more common forms of breast cancer.

• #58 Paget Disease of the Breast | Details, Diagnosis and Signs | American Cancer Society

  https://www.cancer.org/cancer/types/breast-cancer/about/types-of-breast-cancer/paget-disease-of-the-nipple.html

  Paget disease of the breast is a rare type of breast cancer involving the skin of the nipple and the areola (the dark circle around the nipple). […] Paget disease usually affects only one breast. In 80-90% of cases, its usually found along with either ductal carcinoma in situ (DCIS) or infiltrating ductal carcinoma (invasive breast cancer). […] Paget disease of the breast is diagnosed by a biopsy, removing a small piece of the breast tissue and looking at it closely in the lab. In some cases, the entire nipple may be removed. Only a biopsy can show for sure that it is cancer. […] Paget disease can be treated by removing the entire breast (mastectomy) or breast-conserving surgery (BCS) followed by whole-breast radiation therapy. If BCS is done, the entire nipple and areola area also needs to be removed. If invasive cancer is found, the lymph nodes under the arm will be checked for cancer. […] If the cancer has spread within the breast tissue (is invasive), the outlook is not as good, and the cancer will be staged and treated like any other invasive ductal carcinoma.

• #59 Paget Disease of the Breast | Details, Diagnosis and Signs | American Cancer Society

  https://www.cancer.org/cancer/types/breast-cancer/about/types-of-breast-cancer/paget-disease-of-the-nipple.html

  Paget disease of the breast is a rare type of breast cancer involving the skin of the nipple and the areola (the dark circle around the nipple). […] Paget disease usually affects only one breast. In 80-90% of cases, its usually found along with either ductal carcinoma in situ (DCIS) or infiltrating ductal carcinoma (invasive breast cancer). […] Paget disease of the breast is diagnosed by a biopsy, removing a small piece of the breast tissue and looking at it closely in the lab. In some cases, the entire nipple may be removed. Only a biopsy can show for sure that it is cancer. […] Paget disease can be treated by removing the entire breast (mastectomy) or breast-conserving surgery (BCS) followed by whole-breast radiation therapy. If BCS is done, the entire nipple and areola area also needs to be removed. If invasive cancer is found, the lymph nodes under the arm will be checked for cancer. […] If the cancer has spread within the breast tissue (is invasive), the outlook is not as good, and the cancer will be staged and treated like any other invasive ductal carcinoma.

• #60 Paget disease of the breast | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/breast/what-is-breast-cancer/cancerous-tumours/paget-disease-of-the-breast

  Paget disease of the breast is a rare type of breast cancer. It develops as a rash or other skin changes on the nipple, usually on only one breast. […] Most women with Paget disease also have invasive ductal carcinoma or ductal carcinoma in situ (DCIS). The cancer can then spread to the dark-coloured skin around the nipple (called the areola). […] If you have Paget disease of the breast, your healthcare team will create a treatment plan just for you. Surgery is usually the main treatment. You may be offered other treatments based on whether or not you have invasive ductal carcinoma or DCIS and if the cancer has spread to lymph nodes. These treatments may include radiation therapy, hormonal therapy, chemotherapy or targeted therapy.

• #61 Paget’s disease of the breast: Causes, symptoms, treatment, and more

  https://www.medicalnewstoday.com/articles/192362

  When Pagets disease of the breast occurs due to an underlying cancer, the aggressiveness of the cancer depends on the type a person has. […] Usually, Pagets disease of the breast is due to DCIS. With treatment, the outlook for most people with DCIS is very good. […] When Pagets disease is a result of more advanced or aggressive cancers, especially invasive ductal carcinoma, there is a higher mortality rate. […] The factor that plays the most significant role in determining the outcome is early diagnosis. By the time there is a large mass or the cancer has spread to lymph nodes, the disease is more advanced and has a worse prognosis. […] According to the American Cancer Society, Pagets disease usually appears in one breast or nipple. […] Cancer Research UK indicates that about half of all people with Pagets disease of the breast also have a breast lump behind the nipple. This usually means that a person has invasive breast cancer.

• #62 Paget’s disease of the breast: Causes, symptoms, treatment, and more

  https://www.medicalnewstoday.com/articles/192362

  When Pagets disease of the breast occurs due to an underlying cancer, the aggressiveness of the cancer depends on the type a person has. […] Usually, Pagets disease of the breast is due to DCIS. With treatment, the outlook for most people with DCIS is very good. […] When Pagets disease is a result of more advanced or aggressive cancers, especially invasive ductal carcinoma, there is a higher mortality rate. […] The factor that plays the most significant role in determining the outcome is early diagnosis. By the time there is a large mass or the cancer has spread to lymph nodes, the disease is more advanced and has a worse prognosis. […] According to the American Cancer Society, Pagets disease usually appears in one breast or nipple. […] Cancer Research UK indicates that about half of all people with Pagets disease of the breast also have a breast lump behind the nipple. This usually means that a person has invasive breast cancer.

• #63 Paget’s disease of the breast: Causes, symptoms, treatment, and more

  https://www.medicalnewstoday.com/articles/192362

  When Pagets disease of the breast occurs due to an underlying cancer, the aggressiveness of the cancer depends on the type a person has. […] Usually, Pagets disease of the breast is due to DCIS. With treatment, the outlook for most people with DCIS is very good. […] When Pagets disease is a result of more advanced or aggressive cancers, especially invasive ductal carcinoma, there is a higher mortality rate. […] The factor that plays the most significant role in determining the outcome is early diagnosis. By the time there is a large mass or the cancer has spread to lymph nodes, the disease is more advanced and has a worse prognosis. […] According to the American Cancer Society, Pagets disease usually appears in one breast or nipple. […] Cancer Research UK indicates that about half of all people with Pagets disease of the breast also have a breast lump behind the nipple. This usually means that a person has invasive breast cancer.

• #64 Paget’s disease of the breast: Causes, symptoms, treatment, and more

  https://www.medicalnewstoday.com/articles/192362

  When Pagets disease of the breast occurs due to an underlying cancer, the aggressiveness of the cancer depends on the type a person has. […] Usually, Pagets disease of the breast is due to DCIS. With treatment, the outlook for most people with DCIS is very good. […] When Pagets disease is a result of more advanced or aggressive cancers, especially invasive ductal carcinoma, there is a higher mortality rate. […] The factor that plays the most significant role in determining the outcome is early diagnosis. By the time there is a large mass or the cancer has spread to lymph nodes, the disease is more advanced and has a worse prognosis. […] According to the American Cancer Society, Pagets disease usually appears in one breast or nipple. […] Cancer Research UK indicates that about half of all people with Pagets disease of the breast also have a breast lump behind the nipple. This usually means that a person has invasive breast cancer.

• #65 Paget’s Disease of the Breast: Symptoms, Treatment & Prognosis

  https://www.cancercenter.com/cancer-types/breast-cancer/types/rare-breast-cancer-types/pagets-disease-of-the-breast

  Pagets disease of the breast affects about 1 to 4 percent of breast cancer patients, according to the National Cancer Institute (NCI). […] In most cases, people with Pagets disease also have ductal carcinoma in situ (DCIS), which is a breast cancer contained within the milk ducts, or invasive breast cancer. However, Pagets disease is different from other breast cancers in that the cancerous cells are found in the skin of the breast, instead of within the breast. […] Surgery is the primary treatment option for Pagets disease. […] A minority of patients with Paget’s disease have cancer confined to the nipple itself, with no sign of cancer elsewhere in the breast. […] During surgery for a Pagets disease patient also has invasive breast cancer, doctors routinely check the lymph nodes to ensure there are no cancer cells there that require removal. […] Survival rates differ depending on the severity of Pagets disease. The five-year relative survival rate for women with Pagets disease in the United States between 1988 and 2001 was 82.6 percent, according to the NCI.

• #66 Mammary Paget’s Disease: An Update

  https://www.mdpi.com/2072-6694/14/10/2422

  Mammary Paget’s Disease is a non-invasive cutaneous malignancy of the breast involving the nipple-areolar complex that is commonly mistaken for benign breast conditions, leading to delay in diagnosis. […] This review article discusses Paget’s disease etiology, clinical presentation, differential diagnosis, diagnostic work-up, natural history and prognosis. […] Two main theories have been proposed for how MPD arises: the epidermotropic theory and the intraepidermal origin theory. The epidermotropic theory states that Paget cells originate from underlying intraductal carcinoma cells that migrated along the basement membrane into the nipple. This theory is supported by the high incidence of MPD with underlying ductal carcinoma in situ (DCIS). […] The intraepidermal origin theory states that Paget cells result from malignant transformation of pluripotent keratinocyte stem cells or cells of apocrine gland ducts in the absence of underlying carcinoma, which also provides an explanation for cases of MPD where the nipple is spared. […] A hybrid theory proposes that Paget cells can originate either epidermotrophically or intraepidermally, depending on the circumstances and local conditions.

• #67 Paget’s disease of the breast – VALINTERMED treatment in Valencia

  https://valintermed.com/en/medlibrary/disease-of-the-mammary-gland/

  Paget’s disease of the breast (PDMB) is a rare disorder characterized by erythema, eczema-like changes of the skin around the nipple, which may be accompanied by itching, redness, swelling and discharge. […] PDMB is usually associated with invasive breast cancer, which requires special attention and diagnostics for an accurate assessment of the patient’s condition. […] One of the significant contributions to the study of PPD was made in the 1980s, when it was established that skin changes can be associated with extravasation of malignant cells from the internal activity of the mammary gland. […] In recent years, studies have identified several genes associated with the development of Paget’s disease. The most significant are the BRCA1 and BRCA2 genes, which are responsible for DNA repair and regulate the cell cycle. Mutations in these genes significantly increase the risk of developing both breast cancer and BPC. In addition, the TP53 gene, which is responsible for the control of apoptosis and cell division, also plays an important role in the pathogenesis of this disease. […] Treatment of Paget’s disease of the breast requires a multidisciplinary approach. The overall strategy is a comprehensive assessment of the patient’s condition, including surgical removal of the tumor, radiation therapy, and pharmacological therapy.

• #68 Annals of Clinical & Laboratory Science

  https://www.annclinlabsci.org/content/39/1/17.full

  These results suggest that different molecular mechanisms may be involved for MPD depending on the types of underlying lesions, and that different panels of markers should be used to predict MPD depending on its underlying lesions. […] Second, HER2 over-expression and HER2 subtype seem to differ between MPD-associated DCIS and MPD-associated IDC. […] These observations suggest that HER2 over-expression and HER2 subtype are not interchangeable, and over-expression of HER2 and its relationship with the expression of ER and PR may be important in the pathogenesis of MPD. […] Third, MPD and non-MPD nipple-involved tumors, although both have nipple involvement, are subgroups with very different expression patterns and subtype distribution, suggesting that distinct mechanisms are involved in their pathogenesis. […] In summary, the significant differences in ER expression, PR expression, and subtype distribution between MPD associated with two underlying lesions (DCIS and IDC) suggest that different panels of markers should be used to predict MPD in these two lesions.

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