Materiały źródłowe

• #1 Paget Disease of the Breast – NCI

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

  Paget disease of the breast occurs in both women and men, but most cases occur in women. Approximately 1 to 4 percent of all cases of breast cancer also involve Paget disease of the breast. The average age at diagnosis is 57 years, but the disease has been found in adolescents and in people in their late 80s. […] According to NCIs 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. This compares with a 5-year relative survival of 87.1 percent for women diagnosed with any type of breast cancer. For women with both Paget disease of the breast and invasive cancer in the same breast, the 5-year relative survival declined with increasing stage of the cancer (stage I, 95.8 percent; stage II, 77.7 percent; stage III, 46.3 percent; stage IV, 14.3 percent).

• #1 Paget’s Disease of the Nipple | Magee-Women’s Hospital

  https://www.upmc.com/locations/hospitals/magee/services/magee-womens-cancers/breast-cancer-program/types-of-breast-cancer/pagets-disease

  Pagets disease of the nipple is a rare type of breast cancer affecting the nipple and the areola (the circle of skin surrounding the nipple). […] Pagets disease is very rare. Between 1% and 4% of all breast cancer cases involve Pagets disease. […] Anyone can get Paget’s disease of the breast. It mainly impacts women, but men can develop it, too. […] Left untreated, breast cancer can spread and lead to serious health issues and even death. […] The five-year survival rate for people with Pagets disease of the nipple is more than 80%. That’s about the same as the survival rate for people diagnosed with any form of breast cancer. […] Doctors treat Pagets disease with surgery. They may also recommend other cancer treatments, like radiation. […] Surgery is almost always used to treat Pagets disease. […] Doctors often treat Paget’s disease with radiation therapy, which uses high-energy rays to destroy cancer cells.

• #1 Mammary Paget Disease – StatPearls – NCBI Bookshelf

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

  The Presentation of PDB is much less common as compared to the other presentations of breast malignancy like palpable mass or mammographic findings, accounting for about 1-4 percent of overall presentations. […] Although this is an uncommon presentation, any patient with a chronic persistent skin rash of the nipple should be evaluated for PDB. There is some epidemiological data that suggests that the incidence of PDB is decreasing over time. […] Although the overall incidence was high in the data between 1988-2002, according to one estimation provided by the surveillance, epidemiology, and end results (SEER) database, the incidence is decreased by 45% after that due to unknown reasons. This decreasing incidence was greatest for PDB associated with invasive cancer or DCIS.

• #1 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Mammary Paget disease (MPD) is a rare condition primarily affecting adult women, characterized by unilateral skin changes in the nipple–areolar complex (NAC) and frequently associated with underlying breast carcinoma. […] The prevalence of an associated cancer ranges from 67% to 100% with most studies reporting a concurrent malignancy in over 90% of patients. […] MPD represents approximately 1–3% of all BC cases with a prevalence among postmenopausal women aged 50 to 60 years, although it can occur in both sexes at any age. […] The incidence of MPD varies geographically with higher rates reported in Western countries, probably related to lifestyle. […] An intriguing observation is the gradual decline in MPD cases from the 2000s, potentially attributed to an increased detection of early DCIS cases through mammographic screenings.

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

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

  This is a rare disease that corresponds to 1-4.3% of all breast cancers and is frequently associated with intraductal, in situ or invasive neoplasms. […] It is more prevalent in postmenopausal women, usually after the sixth decade of life, but it has also been reported in adolescent and elderly patients. […] It may affect male patients, albeit more rarely. […] Between 93-100% of MPD cases are associated with underlying breast cancer, usually central and multifocal tumors, mainly located near the areola. […] When there is a palpable breast mass, almost always the carcinoma is invasive and associated with high rates of axillary lymph node metastases. […] A careful clinical examination and imaging exams assessment are necessary for diagnostic conclusion. […] Mammograms can detect masses or calcifications that represent invasive or in situ ductal carcinoma, especially when these lesions are palpable.

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

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

  Pagets disease of the breast is a rare type of breast cancer. […] According to the National Health Service in the United Kingdom, about 1% to 4% of females with breast cancer have Pagets disease of the breast. […] The symptoms of Pagets disease of the breast can resemble those of a skin condition, such as eczema or psoriasis. This sometimes delays diagnosis, increasing the risk of the condition advancing. […] 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. […] Only 8% of people with Pagets disease of the breast have no other underlying cancer. […] 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.

• #1 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Specific risk factors for MPD are not identified. […] The same risk conditions known for BC are also applicable to MPD, including advanced age, family history of BC, inherited genetic mutations (BRCA 1 and/or BRCA 2), hormonal exposure (as hormone-replacement therapy and prolonged use of oral contraceptives), obesity, alcohol consumption, and prior thoracic irradiation, especially in women of a young age with high dosages of exposure. […] A careful and timely evaluation, including clinical examination with imaging techniques integration (as DM, US, and MRI), is always required in all patients with NAC skin changes to exclude MPD. […] However, a conclusive diagnosis invariably necessitates histological scrutiny. […] MRI’s higher sensitivity in evaluating the retroareolar region provides crucial information for clinically evident MPD cases with occult findings in DM and US and for the preoperative assessment of disease extent in patients eligible for breast-conserving therapy.

• #1 Surgical treatment in Paget’s disease with invasive ductal carcinoma: an observational study based on SEER | Scientific Reports

  https://www.nature.com/articles/srep45510

  Pagets disease (PD) of the breast is rare, accounting for 13% of all breast malignancies. Pagets disease is characterized by the eczematous eruption and ulceration of the nipple or areola. Due to its rare occurrence, diagnosis of PD may be a delayed or misdiagnosed as benign dermatosis. Breast imaging examination should be used to identify the presence of concomitant breast cancer, and nipple skin biopsy may provide further definitive diagnosis. […] In the majority of patients, PD has been found in association with invasive breast cancer or ductal carcinoma in situ, with concomitant disease identified in up to 82% to 93%. Additionally, several studies have found Pagets disease with invasive ductal carcinoma (PD-IDC) to be associated with tumours that are larger in size and higher in grade as well as negative oestrogen receptor (ER) and progesterone receptor (PR) but positive human epidermal growth factor receptor 2 (Her2) status. Further, diagnosis of PD-IDC may be associated with reduced survival.

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

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

  The outlook for Pagets disease of the breast will depend on various factors, such as whether there is a tumor, which type of cancer a person has, and social and environmental factors. […] According to data from 2000 to 2013, the overall 5-year survival rate for Pagets disease of the breast with DCIS was 97.5%. […] The overall survival rate after 5 years for those with a different type of underlying cancer, concomitant infiltrating duct carcinoma, was less, at 84.1%. […] Pagets disease of the breast is not usually an aggressive breast cancer. However, it can spread if a person does not seek prompt treatment. […] With early diagnosis and treatment, Pagets disease of the breast is typically curable.

• #1 Patients with Nipple-Areola Paget’s Disease and Underlying Invasive Breast Carcinoma Have Very Poor Survival: A Matched Cohort Study | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0061455

  Pagets disease (PD) of the breast is a rare disease. The survival rate of PD was reported to depend on the characteristics of the underlying carcinoma. This study aimed to investigate the characteristics and survival rate of PD patients with underlying invasive breast carcinoma (IBC). Fifty-two patients were diagnosed with PD and an associated IBC from 2001 to 2005 in Fudan University Shanghai Cancer Center. […] The prevalence of an associated cancer ranges from 67-100%, with most studies reporting over 90%. […] Three prognostic factors for PD have been reported in different studies to date: (1) a palpable mass on presentation; (2) an underlying invasive carcinoma of the breast; and (3) the status of the axillary lymph nodes (ALN). […] The survival rate for PD with carcinoma in situ is better than that for PD with invasive carcinoma.

• #1 Paget’s disease of the breast | Breast cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/breast-cancer/types/pagets-disease-breast

  Paget’s disease of the breast is a rare condition of the nipple that is associated with some breast cancers. […] Paget’s disease of the breast is rare. It is found in 1 to 3 out of every 100 breast cancers (1 to 3%) diagnosed in women. It can also happen in men but this is even more rare. […] You usually have regular check ups after treatment for Pagets disease of the breast. How often you have check ups depends on your individual situation.

• #1 ClinMed International Library | Incidental Paget’s Disease of the Nipple in a Prophylactic Skin-Sparing Mastectomy for BRCA1 Mutation: Implications for Increased Use of Prophylactic Nipple Sparing Mastectomy | International Journal of Cancer and Clinica

  http://clinmedjournals.org/articles/ijccr/international-journal-of-cancer-and-clinical-research-ijccr-3-044.php?jid=ijccr

  Incidental Paget’s disease in prophylactic mastectomy specimens raises the concern for continued long term follow-up of high risk patients undergoing NSM. […] Paget’s disease of the nipple is an uncommon presentation of breast cancer and usually presents with nipple excoriation or ulceration with or without an underlying invasive cancer, but it can be found incidentally on mastectomy specimens performed for invasive cancer. […] Increased genetic testing in unaffected women who pursue prophylactic mastectomy may lead to more detection of incidental malignancies even in the era of breast MRI screening. […] Although uncommon, the incidental pathologic finding of Paget’s disease in the nipple raises the importance of continued clinical follow up in high risk patients who increasingly undergo nipple sparing mastectomy.

• #1

  https://www.nhs.uk/conditions/pagets-disease-nipple/

  Paget’s disease of the nipple, also known as Paget’s disease of the breast, is a rare condition associated with breast cancer. […] About 1 to 4% of women with breast cancer have Paget’s disease of the nipple. It can also affect men, but this is extremely rare. […] As Paget’s disease of the nipple is associated with breast cancer, the sooner it’s diagnosed, the better the outcome is likely to be. […] If Paget’s disease is detected and treated in its early stages, there’s a good chance of a full recovery.

• #1 A rare presentation of mammary Paget’s disease involving the entire breast in the absence of any underlying ductal malignancy – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/a-rare-presentation-of-mammary-pagets-disease-involving-the-entire-breast-in-the-absence-of-any-underlying-ductal-malignancy/

  Paget’s disease of the breast is an uncommon form of breast cancer presenting as an eczematous eruption over the nipple and/or areola. […] Most of the cases have an associated malignancy of the underlying breast tissue. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] Pagets disease of the breast is an uncommon form of breast cancer, which presents as an eczematous eruption over the nipple and/or areola. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] An underlying invasive or non-invasive ductal carcinoma has been reported in 85-95% cases of mammary Pagets disease. […] Moreover, significant correlation has been found between the clinical presentation and the underlying disease, more severe cases have a higher probability of underlying malignancy. […] Pagets disease of the breast remains a diagnostic enigma because of its close resemblance to benign skin disorders. […] Any chronic eczematous lesion over the breast should be evaluated thoroughly both clinically as well as histologically to rule out malignancy.

• #1 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 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. […] 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. […] 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.

• #1 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  MPD prognosis in patients without a palpable mass is generally good with a survival rate of 5 years in 90–100% of cases; conversely, women with MPD and a palpable mass associated generally have an underlying invasive carcinoma and a 5-year survival rate of approximately 20–65%. […] Potential advancements in managing patients with MPD offer hopeful opportunities for early diagnosis and increased treatment efficacy. […] These emerging techniques offer hope for early detection and improved patient care, though further validation through large-scale clinical trials is necessary.

• #1 Surgical treatment in Paget’s disease with invasive ductal carcinoma: an observational study based on SEER | Scientific Reports

  https://www.nature.com/articles/srep45510

  Despite these limitations, our study demonstrated that PD-IDC appears to alter the association between prognosis and HER2 status. Meanwhile, BCS with radiotherapy may be a feasible treatment alternative, as it resulted in survival rates similar to those achieved with mastectomy, and SLNB should be considered as an appropriate treatment for patients with PD-IDC. However, surgical treatment plans should be selected based on the results of clinical and imaging assessments. Further studies are needed to minimize variation in treatment of PD-IDC and to establish a standardized management approach for PD-IDC.

• #2 Mammary Paget Disease – StatPearls – NCBI Bookshelf

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

  The Presentation of PDB is much less common as compared to the other presentations of breast malignancy like palpable mass or mammographic findings, accounting for about 1-4 percent of overall presentations. […] Although this is an uncommon presentation, any patient with a chronic persistent skin rash of the nipple should be evaluated for PDB. There is some epidemiological data that suggests that the incidence of PDB is decreasing over time. […] Although the overall incidence was high in the data between 1988-2002, according to one estimation provided by the surveillance, epidemiology, and end results (SEER) database, the incidence is decreased by 45% after that due to unknown reasons. This decreasing incidence was greatest for PDB associated with invasive cancer or DCIS.

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

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

  Most patients diagnosed with Paget’s disease of the breast are over age 50, but rare cases have been diagnosed in patients in their 20s. The average age at diagnosis is 57. The disease is rare among both women and men, but more common in women.

• #2 Paget Disease of the Breast – NCI

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

  Paget disease of the breast occurs in both women and men, but most cases occur in women. Approximately 1 to 4 percent of all cases of breast cancer also involve Paget disease of the breast. The average age at diagnosis is 57 years, but the disease has been found in adolescents and in people in their late 80s. […] According to NCIs 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. This compares with a 5-year relative survival of 87.1 percent for women diagnosed with any type of breast cancer. For women with both Paget disease of the breast and invasive cancer in the same breast, the 5-year relative survival declined with increasing stage of the cancer (stage I, 95.8 percent; stage II, 77.7 percent; stage III, 46.3 percent; stage IV, 14.3 percent).

• #2

  https://www.nursingcenter.com/journalarticle?Article_ID=5555113&Journal_ID=849729&Issue_ID=5555089

  Paget’s disease of the breast (PDB) is a rare disorder of the nipple-areola complex that may present clinically as a unilateral, eczematous, or ulcerated papule or plaque. […] Only 1%-4% of patients with breast cancer also present with PDB. […] However, 92%-100% of PDB cases are associated with ductal carcinoma in situ and/or IDC. […] PDB involving the nipple has been commonly associated with IDC and is thought to be because of epidermal extension of an underlying ductal breast carcinoma. […] Cutaneous involvement is a primary feature of PDB, which makes it distinct from more common types of breast cancer. […] PDB may simulate an eczematous dermatitis, which commonly leads to a delayed diagnosis. […] PDB should be suspected in any patient with chronic, cutaneous changes of the breast that do not respond promptly to topical treatments.

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

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

  This is a rare disease that corresponds to 1-4.3% of all breast cancers and is frequently associated with intraductal, in situ or invasive neoplasms. […] It is more prevalent in postmenopausal women, usually after the sixth decade of life, but it has also been reported in adolescent and elderly patients. […] It may affect male patients, albeit more rarely. […] Between 93-100% of MPD cases are associated with underlying breast cancer, usually central and multifocal tumors, mainly located near the areola. […] When there is a palpable breast mass, almost always the carcinoma is invasive and associated with high rates of axillary lymph node metastases. […] A careful clinical examination and imaging exams assessment are necessary for diagnostic conclusion. […] Mammograms can detect masses or calcifications that represent invasive or in situ ductal carcinoma, especially when these lesions are palpable.

• #2

  https://www.advocatehealth.com/health-services/cancer-institute/cancers-we-treat/breast-cancer/pagets-disease-breast

  Paget’s disease of the breast, often referred to as Pagets disease of the nipple, is a rare type of breast cancer that affects the skin on your nipple. […] Paget’s disease of the breast is a rare form of breast cancer that primarily affects the skin of the nipple and often spreads to the areola, the darker area around the nipple. This condition can easily be mistaken for more benign skin issues like eczema or dermatitis. […] However, Paget’s disease of the breast often indicates an underlying breast cancer, such as ductal carcinoma in situ (DCIS) or invasive breast cancer. […] Paget’s disease of the breast is uncommon, but certain factors may increase your risk, such as age, history of breast cancer, genetic mutations, radiation exposure, and hormone replacement therapy (HRT). […] Diagnosing Paget’s disease of the breast involves several steps. Your doctor will perform a series of tests to confirm the diagnosis, including clinical breast exam, mammogram, ultrasound, and biopsy. […] Treatment for Paget’s disease of the breast typically involves addressing both the disease in the nipple area and any underlying breast cancer.

• #2 Mammary Paget disease. Paget disease of the nipple

  https://dermnetnz.org/topics/mammary-paget-disease

  Mammary Paget disease is an uncommon form of breast cancer, comprising 13% of all breast cancer presentations. […] Mammary Paget disease mainly affects women in their 50s and 60s, with a wide age range reported from adolescence to the elderly. […] Mammary Paget disease is almost always a skin sign of an underlying breast cancer. This may be an in-situ or invasive intraductal carcinoma. Metastases to lymph nodes and beyond may be present. […] Despite the visible lesion, a delay in presentation or diagnosis is common. On average, diagnosis is made 12 months after onset in women and 89 months in men. Prognosis is therefore not as good as for more common forms of breast cancer. It is particularly poor for males, with a 20-30% five-year survival.

• #2 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  MPD prognosis in patients without a palpable mass is generally good with a survival rate of 5 years in 90–100% of cases; conversely, women with MPD and a palpable mass associated generally have an underlying invasive carcinoma and a 5-year survival rate of approximately 20–65%. […] Potential advancements in managing patients with MPD offer hopeful opportunities for early diagnosis and increased treatment efficacy. […] These emerging techniques offer hope for early detection and improved patient care, though further validation through large-scale clinical trials is necessary.

• #2 ClinMed International Library | Incidental Paget’s Disease of the Nipple in a Prophylactic Skin-Sparing Mastectomy for BRCA1 Mutation: Implications for Increased Use of Prophylactic Nipple Sparing Mastectomy | International Journal of Cancer and Clinica

  http://clinmedjournals.org/articles/ijccr/international-journal-of-cancer-and-clinical-research-ijccr-3-044.php?jid=ijccr

  Incidental Paget’s disease in prophylactic mastectomy specimens raises the concern for continued long term follow-up of high risk patients undergoing NSM. […] Paget’s disease of the nipple is an uncommon presentation of breast cancer and usually presents with nipple excoriation or ulceration with or without an underlying invasive cancer, but it can be found incidentally on mastectomy specimens performed for invasive cancer. […] Increased genetic testing in unaffected women who pursue prophylactic mastectomy may lead to more detection of incidental malignancies even in the era of breast MRI screening. […] Although uncommon, the incidental pathologic finding of Paget’s disease in the nipple raises the importance of continued clinical follow up in high risk patients who increasingly undergo nipple sparing mastectomy.

• #2 A rare presentation of mammary Paget’s disease involving the entire breast in the absence of any underlying ductal malignancy – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/a-rare-presentation-of-mammary-pagets-disease-involving-the-entire-breast-in-the-absence-of-any-underlying-ductal-malignancy/

  Paget’s disease of the breast is an uncommon form of breast cancer presenting as an eczematous eruption over the nipple and/or areola. […] Most of the cases have an associated malignancy of the underlying breast tissue. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] Pagets disease of the breast is an uncommon form of breast cancer, which presents as an eczematous eruption over the nipple and/or areola. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] An underlying invasive or non-invasive ductal carcinoma has been reported in 85-95% cases of mammary Pagets disease. […] Moreover, significant correlation has been found between the clinical presentation and the underlying disease, more severe cases have a higher probability of underlying malignancy. […] Pagets disease of the breast remains a diagnostic enigma because of its close resemblance to benign skin disorders. […] Any chronic eczematous lesion over the breast should be evaluated thoroughly both clinically as well as histologically to rule out malignancy.

• #2 Paget’s Disease | Oncohema Key

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

  Pagets disease, a rare presentation of breast cancer accounts for 1% to 3% of all breast cancers. The majority of cases are associated with an underlying malignancy and prognosis is dependent upon the stage of the underlying cancer. In a recent review of the Surveillance, Epidemiology, and End Results (SEER) data by Chen et al., it was reported that the incidence of Pagets disease has decreased between 1988 and 2002. The age-adjusted incidence rates decreased by 49% for Pagets associated with invasive ductal cancer and by 44% for Pagets associated with ductal carcinoma in situ (DCIS). Pagets disease is a more common pathologic than clinical entity. Its clinical incidence ranges from 0.5% to 2.8% with a mean of 1.3% in more than 50,900 patients combined from nine studies. Histological evidence of Paget cells is present in 0.5% to 4.7% of nipples from breast cancer specimens. In a series by Lagios et. al. of 3,000 consecutive breast cancer mastectomy specimens, 21 (0.7%) had clinical evidence of Pagets disease and 147 (4.9%) had Paget cells histologically, thus yielding a sevenfold difference. Of the 158,621 microscopically confirmed female and male breast cancer registrants from the SEER registry of the National Cancer Institute, 1,775 (1.1%) had histologic Pagets disease. In an update of the SEER data, Pagets disease associated with both invasive and DCIS has decreased from 1988 to 2002 by 49% and 44%, respectively. However the number of cases of Pagets diagnosed without underlying invasive disease may be increasing. This may be accounted for the increased use of mammography and finding cancers at an earlier stage before they develop Pagetoid features. Pagets disease is not confined to women and can occur in men but it is extremely rare. There are probably less than 50 cases described in the world medical literature and most are case reports. The majority of patients with Pagets disease present with eczema or ulceration of the nipple and many have a prolonged period of symptoms before diagnosis. It is, therefore, important to have a high index of suspicion for Pagets when a patient presents with nipple complaints. The clinical differential diagnosis of scaling skin and erythema of the nipple-areola complex in addition to Pagets includes eczema, contact dermatitis, and post radiation dermatitis. The diagnosis can be obtained by scrape cytology, a superficial epidermal shave biopsy, a punch biopsy, a wedge incision biopsy, or nipple excision. In patients with clinical Pagets disease, the reported incidence of mammographic findings varies in the literature. For those patients with Pagets without a palpable breast mass, mammography has been reported as normal in 2.5% to 100% of patients.

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

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

  The outlook for Pagets disease of the breast will depend on various factors, such as whether there is a tumor, which type of cancer a person has, and social and environmental factors. […] According to data from 2000 to 2013, the overall 5-year survival rate for Pagets disease of the breast with DCIS was 97.5%. […] The overall survival rate after 5 years for those with a different type of underlying cancer, concomitant infiltrating duct carcinoma, was less, at 84.1%. […] Pagets disease of the breast is not usually an aggressive breast cancer. However, it can spread if a person does not seek prompt treatment. […] With early diagnosis and treatment, Pagets disease of the breast is typically curable.

• #3 Paget disease (breast) | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/paget-disease-breast?lang=us

  Paget disease of the breast, which is also known as Paget disease of the nipple, has traditionally been described as a form of breast malignancy characterized by infiltration of the nipple epidermis by malignant cells. The condition can represent 1-5% of breast malignancies. The average age at diagnosis is around the 6th decade (53-59 years). […] In most cases of Paget of the breast, there are malignant ductal cells that extend to the nipple surface through the terminal lactiferous ducts. Malignant epithelial (Paget) cells infiltrate and proliferate in the epidermis, causing an eczema-like rash of the nipple and the areolar skin. […] Mammary Paget disease can be undetectable on mammography in ~50% of cases. Features that may be evident on mammography include skin thickening, nipple retraction, subareolar or more diffuse malignant microcalcifications, and discrete subareolar mass(es).

• #3 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) […] Relatively uncommon; occurs in 1 – 4% of women and 1 – 2% of men with breast cancer (Breast Cancer Res Treat 2008;111:313) […] Wide age range of 20 – 90 years, with a peak incidence between sixth and seventh decade (mean: 64 years) (Breast Dis 2020;39:119) […] Between 20 – 30% of patients are premenopausal […] Histologic (subclinical) evidence of MPD may be more frequent.

• #3 Paget’s Disease of the Breast – The Operative Review Of Surgery

  https://operativereview.com/pagets-disease-of-the-breast/

  Pagets Indicates an Underlying Ductal Carcinoma In Situ (DCIS) or Invasive Ductal Carcinoma (IDC) in 80-90% of Cases 3. […] 1-4% of All Breast Cancer Patients Also Have Pagets Disease of the Breast 4. […] Most Common in Postmenopausal Women Aged 50-60 5. […] Reported Age Range: 20-90 Years 7. […] Majority in Women (97.9%) But Can Affect Men (2.1%) 7.

• #3 Paget’s Disease | Oncohema Key

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

  Pagets disease, a rare presentation of breast cancer accounts for 1% to 3% of all breast cancers. The majority of cases are associated with an underlying malignancy and prognosis is dependent upon the stage of the underlying cancer. In a recent review of the Surveillance, Epidemiology, and End Results (SEER) data by Chen et al., it was reported that the incidence of Pagets disease has decreased between 1988 and 2002. The age-adjusted incidence rates decreased by 49% for Pagets associated with invasive ductal cancer and by 44% for Pagets associated with ductal carcinoma in situ (DCIS). Pagets disease is a more common pathologic than clinical entity. Its clinical incidence ranges from 0.5% to 2.8% with a mean of 1.3% in more than 50,900 patients combined from nine studies. Histological evidence of Paget cells is present in 0.5% to 4.7% of nipples from breast cancer specimens. In a series by Lagios et. al. of 3,000 consecutive breast cancer mastectomy specimens, 21 (0.7%) had clinical evidence of Pagets disease and 147 (4.9%) had Paget cells histologically, thus yielding a sevenfold difference. Of the 158,621 microscopically confirmed female and male breast cancer registrants from the SEER registry of the National Cancer Institute, 1,775 (1.1%) had histologic Pagets disease. In an update of the SEER data, Pagets disease associated with both invasive and DCIS has decreased from 1988 to 2002 by 49% and 44%, respectively. However the number of cases of Pagets diagnosed without underlying invasive disease may be increasing. This may be accounted for the increased use of mammography and finding cancers at an earlier stage before they develop Pagetoid features. Pagets disease is not confined to women and can occur in men but it is extremely rare. There are probably less than 50 cases described in the world medical literature and most are case reports. The majority of patients with Pagets disease present with eczema or ulceration of the nipple and many have a prolonged period of symptoms before diagnosis. It is, therefore, important to have a high index of suspicion for Pagets when a patient presents with nipple complaints. The clinical differential diagnosis of scaling skin and erythema of the nipple-areola complex in addition to Pagets includes eczema, contact dermatitis, and post radiation dermatitis. The diagnosis can be obtained by scrape cytology, a superficial epidermal shave biopsy, a punch biopsy, a wedge incision biopsy, or nipple excision. In patients with clinical Pagets disease, the reported incidence of mammographic findings varies in the literature. For those patients with Pagets without a palpable breast mass, mammography has been reported as normal in 2.5% to 100% of patients.

• #3 A rare presentation of mammary Paget’s disease involving the entire breast in the absence of any underlying ductal malignancy – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/a-rare-presentation-of-mammary-pagets-disease-involving-the-entire-breast-in-the-absence-of-any-underlying-ductal-malignancy/

  Paget’s disease of the breast is an uncommon form of breast cancer presenting as an eczematous eruption over the nipple and/or areola. […] Most of the cases have an associated malignancy of the underlying breast tissue. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] Pagets disease of the breast is an uncommon form of breast cancer, which presents as an eczematous eruption over the nipple and/or areola. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] An underlying invasive or non-invasive ductal carcinoma has been reported in 85-95% cases of mammary Pagets disease. […] Moreover, significant correlation has been found between the clinical presentation and the underlying disease, more severe cases have a higher probability of underlying malignancy. […] Pagets disease of the breast remains a diagnostic enigma because of its close resemblance to benign skin disorders. […] Any chronic eczematous lesion over the breast should be evaluated thoroughly both clinically as well as histologically to rule out malignancy.

• #3 Paget’s Disease of the Breast

  http://www.aboutcancer.com/pagets_harris.htm

  In 807 patients with clinical Paget’s disease from 12 series, 371 (46%) presented with a breast mass, and 436 (54%) presented without a mass. In patients with a mass, 93% had an invasive breast cancer, and 7% had ductal carcinoma in situ (DCIS). In patients without a mass, 38% had an invasive breast cancer, and 62% had DCIS. […] Of the 158,621 microscopically confirmed female and male breast cancer registrants from the Surveillance Epidemiology and End Results registry of the National Cancer Institute, 1,775 (1.1%) had histologic Paget’s disease. Of breast cancer patients from this database, Paget’s disease was histologically identified in 1.1% of white female patients, 1.3% of African-American female patients, 1.1% of white male patients, and no African-American male patients. […] Paget’s disease is a more common pathologic than clinical entity. Its clinical incidence ranges from 0.5% to 2.6%, with a mean of 1.1% in more than 44,000 patients combined from eight studies. Histologic evidence of Paget’s cells is present in 0.5% to 4.7% of nipples from breast cancer specimens.

• #3 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Specific risk factors for MPD are not identified. […] The same risk conditions known for BC are also applicable to MPD, including advanced age, family history of BC, inherited genetic mutations (BRCA 1 and/or BRCA 2), hormonal exposure (as hormone-replacement therapy and prolonged use of oral contraceptives), obesity, alcohol consumption, and prior thoracic irradiation, especially in women of a young age with high dosages of exposure. […] A careful and timely evaluation, including clinical examination with imaging techniques integration (as DM, US, and MRI), is always required in all patients with NAC skin changes to exclude MPD. […] However, a conclusive diagnosis invariably necessitates histological scrutiny. […] MRI’s higher sensitivity in evaluating the retroareolar region provides crucial information for clinically evident MPD cases with occult findings in DM and US and for the preoperative assessment of disease extent in patients eligible for breast-conserving therapy.

• #3

  https://www.advocatehealth.com/health-services/cancer-institute/cancers-we-treat/breast-cancer/pagets-disease-breast

  Paget’s disease of the breast, often referred to as Pagets disease of the nipple, is a rare type of breast cancer that affects the skin on your nipple. […] Paget’s disease of the breast is a rare form of breast cancer that primarily affects the skin of the nipple and often spreads to the areola, the darker area around the nipple. This condition can easily be mistaken for more benign skin issues like eczema or dermatitis. […] However, Paget’s disease of the breast often indicates an underlying breast cancer, such as ductal carcinoma in situ (DCIS) or invasive breast cancer. […] Paget’s disease of the breast is uncommon, but certain factors may increase your risk, such as age, history of breast cancer, genetic mutations, radiation exposure, and hormone replacement therapy (HRT). […] Diagnosing Paget’s disease of the breast involves several steps. Your doctor will perform a series of tests to confirm the diagnosis, including clinical breast exam, mammogram, ultrasound, and biopsy. […] Treatment for Paget’s disease of the breast typically involves addressing both the disease in the nipple area and any underlying breast cancer.

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

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

  The outlook for Pagets disease of the breast will depend on various factors, such as whether there is a tumor, which type of cancer a person has, and social and environmental factors. […] According to data from 2000 to 2013, the overall 5-year survival rate for Pagets disease of the breast with DCIS was 97.5%. […] The overall survival rate after 5 years for those with a different type of underlying cancer, concomitant infiltrating duct carcinoma, was less, at 84.1%. […] Pagets disease of the breast is not usually an aggressive breast cancer. However, it can spread if a person does not seek prompt treatment. […] With early diagnosis and treatment, Pagets disease of the breast is typically curable.

• #3 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Accurate assessment and appropriate management of patients with MPD require a multidisciplinary discussion conducted by an expert team. […] Following this study, BCS is contra-indicated in MPD treatment. […] Since MPD frequently underlies invasive carcinoma, in accord with the MD Anderson Cancer Center consensus, we can distinguish two conditions for breast surgery treatments: (a) NAC biopsy positive and imaging negative and (b) NAC biopsy positive and imaging positive after further evaluation. […] MPD prognosis is related to invasive components, axillary lymph node metastasis, and palpable mass. […] Invasive cancers associated with MPD are more likely to be high-grade, estrogen- and progesterone-receptor negative, and human epidermal growth factor receptor 2 positive than those in patients with no associated MPD.

• #3 Paget’s Disease of the Breast: A Tertiary Hospital Experience

  https://www.itmedicalteam.pl/articles/pagets-disease-of-the-breast-a-tertiary-hospital-experience-108128.html

  Pagets disease of the breast is rare. It is one of the foremost subtypes of carcinoma of the breast, accounting for only 1-3% of all breast tumors. […] This study aims at evaluating the epidemiology and outcome of surgical treatment of Pagets disease of the breast in Port Harcourt, Nigeria. […] The prevalence of Pagets disease of the breast was found to be 1.14% of all cancers of the breast in our center. […] Pagets disease of the breast is not a very common disease. The prevalence of 1.4% of all breast cancers seen in our center is in line with the 1 4.3% reported by Tavassoli et al. in 1999. […] The high incidence of late presentation and the difficulty of adequate follow-up make it difficult for conservative breast surgeries to be advised in our centre.

• #4 Paget’s disease of the breast | Breast cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/breast-cancer/types/pagets-disease-breast

  Paget’s disease of the breast is a rare condition of the nipple that is associated with some breast cancers. […] Paget’s disease of the breast is rare. It is found in 1 to 3 out of every 100 breast cancers (1 to 3%) diagnosed in women. It can also happen in men but this is even more rare. […] You usually have regular check ups after treatment for Pagets disease of the breast. How often you have check ups depends on your individual situation.

• #4 Update on the surgical management of Paget’s disease – Trebska-McGowan – Gland Surgery

  https://gs.amegroups.org/article/view/2625/html

  Mammary Pagets disease is a rare form of breast neoplasm that is associated with approximately 3% of all breast cancers (1). It is much more frequently found in females than males, though it can be found in men, with some series suggesting worse prognosis in men (2). The vast majority of patients diagnosed with Pagets disease have an associated underlying neoplasm in the breast. In several series, the percentage of patients diagnosed with Pagets disease of the nipple found to have invasive or non-invasive carcinoma is upwards of 90% (1,5-8). Most often Pagets disease is diagnosed in women in the sixth and seventh decade with a mean age of diagnosis reported at 62.6 years (9), but the age distribution is equivalent to that of breast cancer in general, and it may affect younger patients or octogenarians (10). Pagets disease of the breast is a relatively uncommon condition affecting the nipple and the surrounding tissue. Almost universally, it is associated with DCIS or invasive cancer in the ipsilateral breast. Because these underlying malignancies tend to be multifocal and multicentric, mammography often is not sufficient and MRI may be necessary to evaluate the true extent of the disease. Both total mastectomy and breast preserving surgery followed by appropriate adjuvant therapy are acceptable treatment options for carefully selected patients with Pagets disease.

• #4 Mammary Paget disease. Paget disease of the nipple

  https://dermnetnz.org/topics/mammary-paget-disease

  Mammary Paget disease is an uncommon form of breast cancer, comprising 13% of all breast cancer presentations. […] Mammary Paget disease mainly affects women in their 50s and 60s, with a wide age range reported from adolescence to the elderly. […] Mammary Paget disease is almost always a skin sign of an underlying breast cancer. This may be an in-situ or invasive intraductal carcinoma. Metastases to lymph nodes and beyond may be present. […] Despite the visible lesion, a delay in presentation or diagnosis is common. On average, diagnosis is made 12 months after onset in women and 89 months in men. Prognosis is therefore not as good as for more common forms of breast cancer. It is particularly poor for males, with a 20-30% five-year survival.

• #4 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Mammary Paget disease (MPD) is a rare condition primarily affecting adult women, characterized by unilateral skin changes in the nipple–areolar complex (NAC) and frequently associated with underlying breast carcinoma. […] The prevalence of an associated cancer ranges from 67% to 100% with most studies reporting a concurrent malignancy in over 90% of patients. […] MPD represents approximately 1–3% of all BC cases with a prevalence among postmenopausal women aged 50 to 60 years, although it can occur in both sexes at any age. […] The incidence of MPD varies geographically with higher rates reported in Western countries, probably related to lifestyle. […] An intriguing observation is the gradual decline in MPD cases from the 2000s, potentially attributed to an increased detection of early DCIS cases through mammographic screenings.

• #4

  https://www.aurorahealthcare.org/services/cancer/breast-cancer/pagets-disease-breast

  Paget’s disease of the breast, also known as Pagets disease of the nipple, is an extremely rare type of breast cancer that affects the skin on the nipple and breast. […] Pagets disease of the breast typically signals that there might be underlying breast cancer, either in the ducts of the breast (ductal carcinoma in situ) or invasive breast cancer. […] Understanding potential risk factors can help you be more vigilant of your health. While Paget’s disease of the breast is rare, several factors might increase your risk: […] Most cases are diagnosed in women over the age of 50. […] A personal or family history of breast cancer can elevate your risk. […] Certain genetic mutations, such as BRCA1 or BRCA2, can increase your risk. […] Previous radiation treatment to your chest can also be a risk factor.

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

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

  This is a rare disease that corresponds to 1-4.3% of all breast cancers and is frequently associated with intraductal, in situ or invasive neoplasms. […] It is more prevalent in postmenopausal women, usually after the sixth decade of life, but it has also been reported in adolescent and elderly patients. […] It may affect male patients, albeit more rarely. […] Between 93-100% of MPD cases are associated with underlying breast cancer, usually central and multifocal tumors, mainly located near the areola. […] When there is a palpable breast mass, almost always the carcinoma is invasive and associated with high rates of axillary lymph node metastases. […] A careful clinical examination and imaging exams assessment are necessary for diagnostic conclusion. […] Mammograms can detect masses or calcifications that represent invasive or in situ ductal carcinoma, especially when these lesions are palpable.

• #4 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Accurate assessment and appropriate management of patients with MPD require a multidisciplinary discussion conducted by an expert team. […] Following this study, BCS is contra-indicated in MPD treatment. […] Since MPD frequently underlies invasive carcinoma, in accord with the MD Anderson Cancer Center consensus, we can distinguish two conditions for breast surgery treatments: (a) NAC biopsy positive and imaging negative and (b) NAC biopsy positive and imaging positive after further evaluation. […] MPD prognosis is related to invasive components, axillary lymph node metastasis, and palpable mass. […] Invasive cancers associated with MPD are more likely to be high-grade, estrogen- and progesterone-receptor negative, and human epidermal growth factor receptor 2 positive than those in patients with no associated MPD.

• #4

  https://www.nursingcenter.com/journalarticle?Article_ID=5555113&Journal_ID=849729&Issue_ID=5555089

  Treatment and prognosis of PDB depend on the type of underlying associated breast carcinoma and presence or absence of axillary lymph node involvement. […] Patients with PDB should be promptly referred to oncology for staging and treatment. […] This case study highlights the importance of histologic examination via incisional or deep punch biopsy to confirm a diagnosis of PDB, regardless of prior normal mammogram or breast ultrasound. […] Despite high prevalence of underlying breast cancer among patients with PDB, only approximately 35%-65% of patients with biopsy-proven PDB exhibit findings on mammography that are concerning for underlying breast carcinoma. […] Mammography has low sensitivity to detect PDB and should be used as a complimentary diagnostic tool to surgical wedge or punch biopsy when PDB is suspected.

• #5 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). […] This condition occurs most often in women, and the average age at diagnosis is 57, though it may affect anyone from teens up to people in their late 80s. […] As many as half of Pagets disease patients also have a palpable breast lump, according to the NCI, so a physical breast exam may be a part of the diagnosis process. […] 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.

• #5 Patients with Nipple-Areola Paget’s Disease and Underlying Invasive Breast Carcinoma Have Very Poor Survival: A Matched Cohort Study | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0061455

  Pagets disease (PD) of the breast is a rare disease. The survival rate of PD was reported to depend on the characteristics of the underlying carcinoma. This study aimed to investigate the characteristics and survival rate of PD patients with underlying invasive breast carcinoma (IBC). Fifty-two patients were diagnosed with PD and an associated IBC from 2001 to 2005 in Fudan University Shanghai Cancer Center. […] The prevalence of an associated cancer ranges from 67-100%, with most studies reporting over 90%. […] Three prognostic factors for PD have been reported in different studies to date: (1) a palpable mass on presentation; (2) an underlying invasive carcinoma of the breast; and (3) the status of the axillary lymph nodes (ALN). […] The survival rate for PD with carcinoma in situ is better than that for PD with invasive carcinoma.

• #5 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Specific risk factors for MPD are not identified. […] The same risk conditions known for BC are also applicable to MPD, including advanced age, family history of BC, inherited genetic mutations (BRCA 1 and/or BRCA 2), hormonal exposure (as hormone-replacement therapy and prolonged use of oral contraceptives), obesity, alcohol consumption, and prior thoracic irradiation, especially in women of a young age with high dosages of exposure. […] A careful and timely evaluation, including clinical examination with imaging techniques integration (as DM, US, and MRI), is always required in all patients with NAC skin changes to exclude MPD. […] However, a conclusive diagnosis invariably necessitates histological scrutiny. […] MRI’s higher sensitivity in evaluating the retroareolar region provides crucial information for clinically evident MPD cases with occult findings in DM and US and for the preoperative assessment of disease extent in patients eligible for breast-conserving therapy.

• #5

  https://www.nursingcenter.com/journalarticle?Article_ID=5555113&Journal_ID=849729&Issue_ID=5555089

  Treatment and prognosis of PDB depend on the type of underlying associated breast carcinoma and presence or absence of axillary lymph node involvement. […] Patients with PDB should be promptly referred to oncology for staging and treatment. […] This case study highlights the importance of histologic examination via incisional or deep punch biopsy to confirm a diagnosis of PDB, regardless of prior normal mammogram or breast ultrasound. […] Despite high prevalence of underlying breast cancer among patients with PDB, only approximately 35%-65% of patients with biopsy-proven PDB exhibit findings on mammography that are concerning for underlying breast carcinoma. […] Mammography has low sensitivity to detect PDB and should be used as a complimentary diagnostic tool to surgical wedge or punch biopsy when PDB is suspected.

• #6 Paget’s Disease | Breast Cancer 101 | Breastlink

  http://breastlink.com/breast-cancer-101/rare-breast-cancer-types/pagets-disease

  Paget’s disease of the breast accounts for 1-4 percent of breast cancer cases. […] The median age of diagnosis is 57, but cases have been found in patients as young as their early 20s and as old as their late 80s. […] More than 50 percent of patients with Paget’s disease will have an abnormality on their mammogram. […] Careful follow-up is always necessary with a potential diagnosis of Paget’s disease.

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

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

  Pagets disease of the breast is a rare type of breast cancer. […] According to the National Health Service in the United Kingdom, about 1% to 4% of females with breast cancer have Pagets disease of the breast. […] The symptoms of Pagets disease of the breast can resemble those of a skin condition, such as eczema or psoriasis. This sometimes delays diagnosis, increasing the risk of the condition advancing. […] 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. […] Only 8% of people with Pagets disease of the breast have no other underlying cancer. […] 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.

• #6

  https://www.aurorahealthcare.org/services/cancer/breast-cancer/pagets-disease-breast

  Paget’s disease of the breast, also known as Pagets disease of the nipple, is an extremely rare type of breast cancer that affects the skin on the nipple and breast. […] Pagets disease of the breast typically signals that there might be underlying breast cancer, either in the ducts of the breast (ductal carcinoma in situ) or invasive breast cancer. […] Understanding potential risk factors can help you be more vigilant of your health. While Paget’s disease of the breast is rare, several factors might increase your risk: […] Most cases are diagnosed in women over the age of 50. […] A personal or family history of breast cancer can elevate your risk. […] Certain genetic mutations, such as BRCA1 or BRCA2, can increase your risk. […] Previous radiation treatment to your chest can also be a risk factor.

• #7 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) […] Relatively uncommon; occurs in 1 – 4% of women and 1 – 2% of men with breast cancer (Breast Cancer Res Treat 2008;111:313) […] Wide age range of 20 – 90 years, with a peak incidence between sixth and seventh decade (mean: 64 years) (Breast Dis 2020;39:119) […] Between 20 – 30% of patients are premenopausal […] Histologic (subclinical) evidence of MPD may be more frequent.

• #7 A rare presentation of mammary Paget’s disease involving the entire breast in the absence of any underlying ductal malignancy – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/a-rare-presentation-of-mammary-pagets-disease-involving-the-entire-breast-in-the-absence-of-any-underlying-ductal-malignancy/

  Paget’s disease of the breast is an uncommon form of breast cancer presenting as an eczematous eruption over the nipple and/or areola. […] Most of the cases have an associated malignancy of the underlying breast tissue. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] Pagets disease of the breast is an uncommon form of breast cancer, which presents as an eczematous eruption over the nipple and/or areola. […] There have been very few reports wherein the disease has occurred independent of any underlying malignancy. […] An underlying invasive or non-invasive ductal carcinoma has been reported in 85-95% cases of mammary Pagets disease. […] Moreover, significant correlation has been found between the clinical presentation and the underlying disease, more severe cases have a higher probability of underlying malignancy. […] Pagets disease of the breast remains a diagnostic enigma because of its close resemblance to benign skin disorders. […] Any chronic eczematous lesion over the breast should be evaluated thoroughly both clinically as well as histologically to rule out malignancy.

• #7 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Specific risk factors for MPD are not identified. […] The same risk conditions known for BC are also applicable to MPD, including advanced age, family history of BC, inherited genetic mutations (BRCA 1 and/or BRCA 2), hormonal exposure (as hormone-replacement therapy and prolonged use of oral contraceptives), obesity, alcohol consumption, and prior thoracic irradiation, especially in women of a young age with high dosages of exposure. […] A careful and timely evaluation, including clinical examination with imaging techniques integration (as DM, US, and MRI), is always required in all patients with NAC skin changes to exclude MPD. […] However, a conclusive diagnosis invariably necessitates histological scrutiny. […] MRI’s higher sensitivity in evaluating the retroareolar region provides crucial information for clinically evident MPD cases with occult findings in DM and US and for the preoperative assessment of disease extent in patients eligible for breast-conserving therapy.

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

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

  However, a negative exam does not exclude the presence of tumors. […] Some studies report rates of mammographically occult neoplasms ranging from 15 to 65%. […] Ultrasound is particularly useful in cases with negative mammograms. […] Magnetic resonance is highly sensitive for the diagnosis of breast neoplasms, especially if mammography and ultrasound are normal. […] It may demonstrate thickening of the papillary-areolar complex, increase in nipple size, detection of in situ ductal lesions and invasive tumors, even in clinically unsuspected cases. […] Confronted with a diagnosis of MPD, it is essential to investigate mammary gland neoplasm. […] This type of cancer may be in situ or invasive, but it is almost always present. […] Diagnosis is clinical and histopathological. […] The main histological characteristic is the presence of Paget cells, which are arranged in solid groups.

• #8

  https://www.aurorahealthcare.org/services/cancer/breast-cancer/pagets-disease-breast

  Long-term hormone replacement therapy after menopause has been linked to a higher risk of breast cancer. […] Diagnosing Paget’s disease of the breast involves several steps, starting with a thorough examination by your doctor. […] Treating Paget’s disease of the breast typically involves addressing both the disease in the nipple area and any underlying breast cancer.

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

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

  However, a negative exam does not exclude the presence of tumors. […] Some studies report rates of mammographically occult neoplasms ranging from 15 to 65%. […] Ultrasound is particularly useful in cases with negative mammograms. […] Magnetic resonance is highly sensitive for the diagnosis of breast neoplasms, especially if mammography and ultrasound are normal. […] It may demonstrate thickening of the papillary-areolar complex, increase in nipple size, detection of in situ ductal lesions and invasive tumors, even in clinically unsuspected cases. […] Confronted with a diagnosis of MPD, it is essential to investigate mammary gland neoplasm. […] This type of cancer may be in situ or invasive, but it is almost always present. […] Diagnosis is clinical and histopathological. […] The main histological characteristic is the presence of Paget cells, which are arranged in solid groups.

• #9 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Specific risk factors for MPD are not identified. […] The same risk conditions known for BC are also applicable to MPD, including advanced age, family history of BC, inherited genetic mutations (BRCA 1 and/or BRCA 2), hormonal exposure (as hormone-replacement therapy and prolonged use of oral contraceptives), obesity, alcohol consumption, and prior thoracic irradiation, especially in women of a young age with high dosages of exposure. […] A careful and timely evaluation, including clinical examination with imaging techniques integration (as DM, US, and MRI), is always required in all patients with NAC skin changes to exclude MPD. […] However, a conclusive diagnosis invariably necessitates histological scrutiny. […] MRI’s higher sensitivity in evaluating the retroareolar region provides crucial information for clinically evident MPD cases with occult findings in DM and US and for the preoperative assessment of disease extent in patients eligible for breast-conserving therapy.

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

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

  However, a negative exam does not exclude the presence of tumors. […] Some studies report rates of mammographically occult neoplasms ranging from 15 to 65%. […] Ultrasound is particularly useful in cases with negative mammograms. […] Magnetic resonance is highly sensitive for the diagnosis of breast neoplasms, especially if mammography and ultrasound are normal. […] It may demonstrate thickening of the papillary-areolar complex, increase in nipple size, detection of in situ ductal lesions and invasive tumors, even in clinically unsuspected cases. […] Confronted with a diagnosis of MPD, it is essential to investigate mammary gland neoplasm. […] This type of cancer may be in situ or invasive, but it is almost always present. […] Diagnosis is clinical and histopathological. […] The main histological characteristic is the presence of Paget cells, which are arranged in solid groups.

• #10 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Specific risk factors for MPD are not identified. […] The same risk conditions known for BC are also applicable to MPD, including advanced age, family history of BC, inherited genetic mutations (BRCA 1 and/or BRCA 2), hormonal exposure (as hormone-replacement therapy and prolonged use of oral contraceptives), obesity, alcohol consumption, and prior thoracic irradiation, especially in women of a young age with high dosages of exposure. […] A careful and timely evaluation, including clinical examination with imaging techniques integration (as DM, US, and MRI), is always required in all patients with NAC skin changes to exclude MPD. […] However, a conclusive diagnosis invariably necessitates histological scrutiny. […] MRI’s higher sensitivity in evaluating the retroareolar region provides crucial information for clinically evident MPD cases with occult findings in DM and US and for the preoperative assessment of disease extent in patients eligible for breast-conserving therapy.

• #10

  https://www.nhs.uk/conditions/pagets-disease-nipple/diagnosis/

  As Paget’s disease of the nipple is usually a sign of breast cancer, it’s very important you see your GP if you notice changes to the tissue or skin of your breast. […] Paget’s disease is a form of breast cancer and the sooner it’s diagnosed, the better the outcome is likely to be. […] If your GP thinks you may have breast cancer, they’ll refer you to a specialist breast clinic for tests. […] A skin biopsy is often used to confirm a diagnosis of Paget’s disease of the nipple. A small tissue sample will be taken from your nipple or the skin around it. The sample will be examined under a microscope and tested to see if it’s cancerous.

• #11 A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives

  https://www.mdpi.com/2072-6694/15/21/5276

  Specific risk factors for MPD are not identified. […] The same risk conditions known for BC are also applicable to MPD, including advanced age, family history of BC, inherited genetic mutations (BRCA 1 and/or BRCA 2), hormonal exposure (as hormone-replacement therapy and prolonged use of oral contraceptives), obesity, alcohol consumption, and prior thoracic irradiation, especially in women of a young age with high dosages of exposure. […] A careful and timely evaluation, including clinical examination with imaging techniques integration (as DM, US, and MRI), is always required in all patients with NAC skin changes to exclude MPD. […] However, a conclusive diagnosis invariably necessitates histological scrutiny. […] MRI’s higher sensitivity in evaluating the retroareolar region provides crucial information for clinically evident MPD cases with occult findings in DM and US and for the preoperative assessment of disease extent in patients eligible for breast-conserving therapy.

• #12

  https://www.aurorahealthcare.org/services/cancer/breast-cancer/pagets-disease-breast

  Paget’s disease of the breast, also known as Pagets disease of the nipple, is an extremely rare type of breast cancer that affects the skin on the nipple and breast. […] Pagets disease of the breast typically signals that there might be underlying breast cancer, either in the ducts of the breast (ductal carcinoma in situ) or invasive breast cancer. […] Understanding potential risk factors can help you be more vigilant of your health. While Paget’s disease of the breast is rare, several factors might increase your risk: […] Most cases are diagnosed in women over the age of 50. […] A personal or family history of breast cancer can elevate your risk. […] Certain genetic mutations, such as BRCA1 or BRCA2, can increase your risk. […] Previous radiation treatment to your chest can also be a risk factor.

Zobacz więcej