In 1874, Sir James Paget, an English surgeon and pathologist observed chronic nipple lesions in fifteen female patients. Each of these women eventually developed breast cancer. These lesions were defined as vesicular or eczematous ulcerative lesions with yellowish transparent exudate.
At first, these epidermal lesions were considered benign, but later it was found that they contained cancer cells. These lesions mainly occur on the areola and the nipple. This disorder was eventually termed breast Paget disease or mammary Paget disease.
Extramammary Paget’s disease is a similar disease process which affects both female and male external genitalia. The histological characteristics of these two disorders are identical, but their etiologies are distinct.
Epidemiology
Presenting with PDB is considerably less common than other breast malignancy presentations such as palpable mass or mammogram findings, which account for 1%-4% of all cases.
While women are typically affected by this illness, men might occasionally exhibit similar symptoms. The typical age range for diagnosis is between 26-88 years, with the highest occurrence among postmenopausal women in their 50s.
Despite the rarity of this manifestation, all individuals with a chronic rash on the nipple should be investigated for mammary Paget Disease. Epidemiological evidence indicates that cases of PDB are on the rise.
Despite the fact that the incidence was quite high between 1988 and 2002, the SEER database estimates that the incidence has reduced by 45% since then due to unexplained causes. This decline was most pronounced for PDB cases linked with invasive cancer.
Anatomy
Pathophysiology
Two theories aim to explain the pathogenesis of this condition.
These are:
Transformation Theory:
The hypothesis posits that PDB emerges from epidermal keratinocytes independent of the underlying breast cancer and is actually an epidermal carcinoma in situ. In 1881, George Thin suggested the notion that breast duct secretions repeatedly injure the epithelium, leading to these keratinocytes to transition into cancer cells.
This notion was backed by the fact that a small proportion of PDB cases did not have parenchymal cancer. The presence of two distinct neoplastic processes was frequently observed when malignancies were present in the nipple. Out of 80 female patients, 29 patients were diagnosed with PDB and breast cancer, the underlying tumour was more than 2cm away from the areolar margin.
Additionally, distinct “pre-Paget cells” with an intermediate appearance between keratinocytes and Pagets cells have been observed, indicating that epidermal cells can acquire the characteristics of ductal cells. This notion has gone out of favour since the involvement of the lactiferous ducts immediately beneath the nasopharynx can typically be proven if the needed biopsies and analyses are performed.
Epidermotropic theory:
According to this theory, the Paget cell arises from a mammary adenocarcinoma that migrates from the breast ductal system to the nipple epidermis. In numerous case series, it was discovered that the immunohistochemistry (IHC) staining properties of Paget cells and ductal epithelial cells were identical.
This was not the case among Paget cells or epidermal keratinocytes surrounding the nipple tissue. Similarly, various molecular markers were identified in Paget cells also present in numerous parenchymal breast cancers, such as HER2 gene amplification or overexpression.
About 85 percent of PDB cases are stained with an anti-HER2 monoclonal antibody. The progression of Paget cells from the duct system to the nipple epidermis is thought to be mediated by HER2 receptor-mediated motility factors.
These findings suggest that Paget cells and the underlying ductal carcinoma share a common genetic mutation and/or a common progenitor cell. Out of the two, the epidermotropic theory is currently more accepted.
Etiology
It has been commonly accepted that PDB is linked with a presentation of breast cancer, typically ductal carcinoma in situ or invasive ductal carcinoma.
The malignant epithelial cells are thought to migrate to the skin via lactiferous ducts and ductules. There are no clearly defined risk factors apart from those shared by other breast cancers.
The risk factors for the same are:
Family history of ovarian or breast cancer, or both
Exposure to radiation (particularly involving the chest)
Hyperplasia
Lobular carcinoma in situ
Identification of dense breast tissue on the mammogram
Genetics
Prognostic Factors
The prognosis of PDB depends on the first manifestation of the disease and the existence of axillary lymph node metastases or invasive ductal carcinoma. If PDB initially manifests with a palpable mass, the disease is typically more advanced than in patients without the presence of a palpable mass.
When there is no palpable breast lump, the 5-year survival rate for patients following excision is 92%, and in the presence of one; it’s 38%. Presence of lymphadenopathy further worsens the prognosis.
Clinical History
Physical Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Hormone Therapy
Immunotherapy
Hyperthermia
Photodynamic Therapy
Stem Cell Transplant
Targeted Therapy
Palliative Care
Medication
Future Trends
Media Gallary
References
https://www.ncbi.nlm.nih.gov/books/NBK563228/
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In 1874, Sir James Paget, an English surgeon and pathologist observed chronic nipple lesions in fifteen female patients. Each of these women eventually developed breast cancer. These lesions were defined as vesicular or eczematous ulcerative lesions with yellowish transparent exudate.
At first, these epidermal lesions were considered benign, but later it was found that they contained cancer cells. These lesions mainly occur on the areola and the nipple. This disorder was eventually termed breast Paget disease or mammary Paget disease.
Extramammary Paget’s disease is a similar disease process which affects both female and male external genitalia. The histological characteristics of these two disorders are identical, but their etiologies are distinct.
Presenting with PDB is considerably less common than other breast malignancy presentations such as palpable mass or mammogram findings, which account for 1%-4% of all cases.
While women are typically affected by this illness, men might occasionally exhibit similar symptoms. The typical age range for diagnosis is between 26-88 years, with the highest occurrence among postmenopausal women in their 50s.
Despite the rarity of this manifestation, all individuals with a chronic rash on the nipple should be investigated for mammary Paget Disease. Epidemiological evidence indicates that cases of PDB are on the rise.
Despite the fact that the incidence was quite high between 1988 and 2002, the SEER database estimates that the incidence has reduced by 45% since then due to unexplained causes. This decline was most pronounced for PDB cases linked with invasive cancer.
Two theories aim to explain the pathogenesis of this condition.
These are:
Transformation Theory:
The hypothesis posits that PDB emerges from epidermal keratinocytes independent of the underlying breast cancer and is actually an epidermal carcinoma in situ. In 1881, George Thin suggested the notion that breast duct secretions repeatedly injure the epithelium, leading to these keratinocytes to transition into cancer cells.
This notion was backed by the fact that a small proportion of PDB cases did not have parenchymal cancer. The presence of two distinct neoplastic processes was frequently observed when malignancies were present in the nipple. Out of 80 female patients, 29 patients were diagnosed with PDB and breast cancer, the underlying tumour was more than 2cm away from the areolar margin.
Additionally, distinct “pre-Paget cells” with an intermediate appearance between keratinocytes and Pagets cells have been observed, indicating that epidermal cells can acquire the characteristics of ductal cells. This notion has gone out of favour since the involvement of the lactiferous ducts immediately beneath the nasopharynx can typically be proven if the needed biopsies and analyses are performed.
Epidermotropic theory:
According to this theory, the Paget cell arises from a mammary adenocarcinoma that migrates from the breast ductal system to the nipple epidermis. In numerous case series, it was discovered that the immunohistochemistry (IHC) staining properties of Paget cells and ductal epithelial cells were identical.
This was not the case among Paget cells or epidermal keratinocytes surrounding the nipple tissue. Similarly, various molecular markers were identified in Paget cells also present in numerous parenchymal breast cancers, such as HER2 gene amplification or overexpression.
About 85 percent of PDB cases are stained with an anti-HER2 monoclonal antibody. The progression of Paget cells from the duct system to the nipple epidermis is thought to be mediated by HER2 receptor-mediated motility factors.
These findings suggest that Paget cells and the underlying ductal carcinoma share a common genetic mutation and/or a common progenitor cell. Out of the two, the epidermotropic theory is currently more accepted.
It has been commonly accepted that PDB is linked with a presentation of breast cancer, typically ductal carcinoma in situ or invasive ductal carcinoma.
The malignant epithelial cells are thought to migrate to the skin via lactiferous ducts and ductules. There are no clearly defined risk factors apart from those shared by other breast cancers.
The risk factors for the same are:
Family history of ovarian or breast cancer, or both
Exposure to radiation (particularly involving the chest)
Hyperplasia
Lobular carcinoma in situ
Identification of dense breast tissue on the mammogram
The prognosis of PDB depends on the first manifestation of the disease and the existence of axillary lymph node metastases or invasive ductal carcinoma. If PDB initially manifests with a palpable mass, the disease is typically more advanced than in patients without the presence of a palpable mass.
When there is no palpable breast lump, the 5-year survival rate for patients following excision is 92%, and in the presence of one; it’s 38%. Presence of lymphadenopathy further worsens the prognosis.
https://www.ncbi.nlm.nih.gov/books/NBK563228/
medtigo
Mammary Paget Disease
Updated :
July 5, 2022
In 1874, Sir James Paget, an English surgeon and pathologist observed chronic nipple lesions in fifteen female patients. Each of these women eventually developed breast cancer. These lesions were defined as vesicular or eczematous ulcerative lesions with yellowish transparent exudate.
At first, these epidermal lesions were considered benign, but later it was found that they contained cancer cells. These lesions mainly occur on the areola and the nipple. This disorder was eventually termed breast Paget disease or mammary Paget disease.
Extramammary Paget’s disease is a similar disease process which affects both female and male external genitalia. The histological characteristics of these two disorders are identical, but their etiologies are distinct.
Presenting with PDB is considerably less common than other breast malignancy presentations such as palpable mass or mammogram findings, which account for 1%-4% of all cases.
While women are typically affected by this illness, men might occasionally exhibit similar symptoms. The typical age range for diagnosis is between 26-88 years, with the highest occurrence among postmenopausal women in their 50s.
Despite the rarity of this manifestation, all individuals with a chronic rash on the nipple should be investigated for mammary Paget Disease. Epidemiological evidence indicates that cases of PDB are on the rise.
Despite the fact that the incidence was quite high between 1988 and 2002, the SEER database estimates that the incidence has reduced by 45% since then due to unexplained causes. This decline was most pronounced for PDB cases linked with invasive cancer.
Two theories aim to explain the pathogenesis of this condition.
These are:
Transformation Theory:
The hypothesis posits that PDB emerges from epidermal keratinocytes independent of the underlying breast cancer and is actually an epidermal carcinoma in situ. In 1881, George Thin suggested the notion that breast duct secretions repeatedly injure the epithelium, leading to these keratinocytes to transition into cancer cells.
This notion was backed by the fact that a small proportion of PDB cases did not have parenchymal cancer. The presence of two distinct neoplastic processes was frequently observed when malignancies were present in the nipple. Out of 80 female patients, 29 patients were diagnosed with PDB and breast cancer, the underlying tumour was more than 2cm away from the areolar margin.
Additionally, distinct “pre-Paget cells” with an intermediate appearance between keratinocytes and Pagets cells have been observed, indicating that epidermal cells can acquire the characteristics of ductal cells. This notion has gone out of favour since the involvement of the lactiferous ducts immediately beneath the nasopharynx can typically be proven if the needed biopsies and analyses are performed.
Epidermotropic theory:
According to this theory, the Paget cell arises from a mammary adenocarcinoma that migrates from the breast ductal system to the nipple epidermis. In numerous case series, it was discovered that the immunohistochemistry (IHC) staining properties of Paget cells and ductal epithelial cells were identical.
This was not the case among Paget cells or epidermal keratinocytes surrounding the nipple tissue. Similarly, various molecular markers were identified in Paget cells also present in numerous parenchymal breast cancers, such as HER2 gene amplification or overexpression.
About 85 percent of PDB cases are stained with an anti-HER2 monoclonal antibody. The progression of Paget cells from the duct system to the nipple epidermis is thought to be mediated by HER2 receptor-mediated motility factors.
These findings suggest that Paget cells and the underlying ductal carcinoma share a common genetic mutation and/or a common progenitor cell. Out of the two, the epidermotropic theory is currently more accepted.
It has been commonly accepted that PDB is linked with a presentation of breast cancer, typically ductal carcinoma in situ or invasive ductal carcinoma.
The malignant epithelial cells are thought to migrate to the skin via lactiferous ducts and ductules. There are no clearly defined risk factors apart from those shared by other breast cancers.
The risk factors for the same are:
Family history of ovarian or breast cancer, or both
Exposure to radiation (particularly involving the chest)
Hyperplasia
Lobular carcinoma in situ
Identification of dense breast tissue on the mammogram
The prognosis of PDB depends on the first manifestation of the disease and the existence of axillary lymph node metastases or invasive ductal carcinoma. If PDB initially manifests with a palpable mass, the disease is typically more advanced than in patients without the presence of a palpable mass.
When there is no palpable breast lump, the 5-year survival rate for patients following excision is 92%, and in the presence of one; it’s 38%. Presence of lymphadenopathy further worsens the prognosis.
https://www.ncbi.nlm.nih.gov/books/NBK563228/
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