Materiały źródłowe

• #1 Paget’s disease of the breast – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-the-breast/diagnosis-treatment/drc-20351084

  If you have Paget’s disease of the breast, you will likely need surgery. The type of surgery depends on the condition of the skin around your nipple and how advanced the underlying cancer is. […] During a total (simple) mastectomy, the surgeon removes the breast tissue, nipple, areola and skin. Other mastectomy procedures may leave some parts of the breast, such as the skin or the nipple. Surgery to create a new breast is optional and can be done at the same time as your mastectomy surgery or it can be done later. […] Surgical options include: […] A mastectomy is an operation to remove all of your breast tissue. Most mastectomy procedures remove all of the breast tissue the lobules, ducts, fatty tissue and some skin, including the nipple and areola (total or simple mastectomy). […] During a lumpectomy, which may be referred to as breast-conserving surgery or wide local excision, the surgeon removes the cancer and a small margin of surrounding healthy tissue. If you and your doctor choose this option, you will also receive radiation therapy afterward.

• #1 Paget Disease of the Breast – NCI

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

  How is Paget disease of the breast treated? […] For many years, mastectomy, with or without the removal of lymph nodes under the arm on the same side of chest (known as axillary lymph node dissection), was regarded as the standard surgery for Paget disease of the breast. […] Studies have shown, however, that breast-conserving surgery that includes removal of the nipple and areola, followed by whole-breast radiation therapy, is a safe option for people with Paget disease of the breast who do not have a palpable lump in their breast and whose mammograms do not reveal a tumor. […] People with Paget disease of the breast who have a breast tumor and are having a mastectomy should be offered sentinel lymph node biopsy to see whether the cancer has spread to the axillary lymph nodes. […] Depending on the stage and other features of the underlying breast tumor (for example, the presence or absence of lymph node involvement, estrogen and progesterone receptors in the tumor cells, and HER2 protein overexpression in the tumor cells), adjuvant therapy, consisting of chemotherapy and/or hormonal therapy, may also be recommended.

• #1 Practical consensus recommendatons for Paget’s disease in breast cancer

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5909301/

  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. Diagnosis and treatment of Paget’s disease is controversial. Expert oncologists discuss on the update on the approaches of Paget’s disease diagnosis and its treatment options. The surgical treatment of Paget’s disease is controversial. Mastectomy with or without axillary lymph node dissection has long been regarded as the standard therapy for Paget’s disease, even in the absence of other clinical signs of malignancy. The underlying mass is often invasive cancer with a high risk of axillary node metastases. The patients have a poorer prognosis and usually the appropriate therapy is based on the pathologic findings of the mass and axillary staging. In the literature, almost all patients treated with local excision, radiotherapy, or both had the clinical presentation of Paget’s disease without a palpable mass. Dixon et al. reported a high recurrence rate of 40% in patients treated with local excision alone, whose mammograms suggested in situ changes confined to the nipple area. Polgr et al. reported a local recurrence rate of 33% with a median follow-up time of 6 years in 33 patients treated with local excision alone with or without underlying ductal carcinoma in situ confined to the nippleareola complex. They concluded that the local excision alone was not an appropriate treatment for patients with Paget’s disease of the nipple.

• #1

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

  After having breast-conserving surgery, it’s likely you’ll need to have radiotherapy to destroy any remaining cancer cells. […] If you have a mastectomy, you may be able to have reconstructive surgery to recreate your breast. […] If you decide not to have breast reconstruction, you can wear a false breast or breast prosthesis, which is available free on the NHS. […] After your surgery, you may need further treatment if you have invasive breast cancer (where the cancerous cells have spread into other tissue in your breast). […] Other types of treatment for breast cancer include: chemotherapy where powerful medicine is used to destroy cancer cells and prevent them dividing and growing, radiotherapy where controlled doses of high-energy radiation, usually X-rays, are used to destroy cancer cells, targeted therapy if your breast cancer is HER2 positive, targeted therapy, usually a medicine called trastuzumab, can be used to treat the cancer by stopping the effects of HER2 and helping your immune system fight off cancer cells, hormone therapy if your breast cancer is hormone-receptor positive, hormone therapy can be used to treat the cancer by lowering the levels of hormones in your body or stopping their effects.

• #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

  In our series, there was no difference between surgical treatment subgroups. […] The previously reported techniques for BCS in patients with PD have varied widely and include nipple excision and central segmentectomy as well as resection plus radiation. There is a place for BCS in selected patients with PD of the breast, especially those with no mass. […] Similarly, these studies supported the use of BCS with radiotherapy as a feasible alternative for patients with PD-IDC. […] Although SLNB may still be considered a controversial treatment in patients with PD, SLNB has become a common approach in patients with breast cancer and appears to be a feasible treatment option. […] In the present study, patients undergoing SLNB had similar survival to those receiving ALND regardless of surgical mode of their breast cancer treatment.

• #1 Practical consensus recommendatons for Paget’s disease in breast cancer

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5909301/

  With the increasing diagnosis of the early breast cancers and the findings from multiple randomized, prospective trials demonstrating that breast conserving therapy is a feasible alternative for patients with disease limited to the central segment of the breast, the reported techniques for conservative management of Paget’s disease vary widely. These include nipple excision alone (partial or complete), central segmentectomy alone, these resections plus radiation and radiation without resection. Recent reports from several studies with long-term follow-up have shown that breast-conserving surgery is equivalent to mastectomy in terms of overall and disease-free survival in patients with breast cancer. If conservative therapy is adopted, the patient should be followed up carefully with regular mammography. Mastectomy should be reserved for the few occasions, when relapse occurs.

• #1 Paget’s disease of the breast | Breast Cancer Now

  https://breastcancernow.org/about-breast-cancer/diagnosis/types-of-breast-cancer/pagets-disease-of-the-breast/

  Treatment aims to remove the area of Pagets disease from your breast and reduce the risk of it coming back or spreading to other parts of the body. […] Surgery is usually the first treatment for Pagets disease of the breast. Its often the only treatment youll need if you have Pagets disease without DCIS or invasive breast cancer. […] If youve been diagnosed with Pagets disease alone, youre most likely to be offered surgery to remove your nipple and areola. This is known as a central excision. […] You may be offered other types of surgery if you have also been diagnosed with DCIS or invasive breast cancer, such as breast-conserving surgery or a mastectomy. […] Depending on the features of your Pagets disease, you may also be offered radiotherapy after your surgery. Radiotherapy uses high-energy x-rays to destroy cancer cells. Its used to reduce the risk of the cancer coming back in the same breast.

• #1 logo–sylvester

  https://umiamihealth.org/en/sylvester-comprehensive-cancer-center/treatments-and-services/breast-cancer/paget%E2%80%99s-disease-of-the-nipple-

  If you have an invasive cancer in addition to Paget’s disease, your doctor may recommend removing your entire breast, called a mastectomy, to make sure all cancer cells are removed. If you decide you want reconstructive surgery to restore your breast, you can do so as part of your initial surgery or wait until later to have surgery. […] In this surgery, your surgeon removes your entire breast, but leaves your underarm lymph nodes intact. […] With a modified radical mastectomy, your surgeon removes the nipple, areola and all of the breast tissue without removing the underlying muscle. […] Doctors use this minor surgery to determine if cancer has spread beyond a primary tumor into your lymphatic system. A sentinel lymph node is the first place cancer cells may move to when they begin to spread.

• #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

  Current National Comprehensive Cancer Network (NCCN) guidelines encouraged axillary staging in patients with PD-IDC, while axillary assessment was not found to be necessary for PD-DCIS undergoing BCS. Further study regarding the potential benefits of SLNB in patients with PD-IDC is warranted. […] 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.

• #1 Treatment options for Paget’s disease of the nipple | Cancer Australia

  https://www.canceraustralia.gov.au/cancer-types/breast-cancer/treatment-options/treatment-options-pagets-disease-nipple

  Removal of the whole breast (mastectomy) and/or removal of one or more lymph nodes from the armpit may be needed for some patients. […] As the nipple is likely to be removed, there are a number of options to recreate a nipple. These include having a nipple tattoo or having a nipple made from your own tissue. […] For women with Paget’s disease of the nipple, radiotherapy is almost always recommended after breast conserving surgery. […] Radiotherapy is sometimes recommended after mastectomy. […] Radiotherapy uses X-rays (controlled doses of radiation) to destroy cancer cells. […] Radiotherapy is usually given once a day, 5 days a week for 3–6 weeks. […] For some women with Paget’s disease of the nipple, radiotherapy may be the only treatment needed after the biopsy used during diagnosis.

• #1 Practical consensus recommendatons for Paget’s disease in breast cancer

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5909301/

  Use of breast radiation therapy alone for the Paget’s disease of the breast has been reported in limited numbers and with varying results. Current studies suggest that irradiation of the breast tissue to a radical dose may be effective against DCIS. This gives support to the view that selected patients with Paget’s disease of the breast can be treated by radiotherapy as an alternative to radical surgery in selected patients with Paget’s disease confined to the nipple, without clinical or radiological detectable breast tumor. […] Both total mastectomy and breast preserving surgery followed by appropriate adjuvant therapy are acceptable treatment options for carefully selected patients with Paget’s disease. The output of this discussion is to provide the best and effective method of diagnosis of disease and provide best treatment options for patients with Paget’s disease of the breast cancer.

• #1 Paget’s Disease

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

  The results with this breast conserving management suggest a place for radiotherapy in the treatment of Paget’s disease limited to the nipple. […] Radiotherapy is a valuable alternative to mastectomy. […] In this selected group, radical radiotherapy with small fields localised to the involved skin is an effective alternative to mastectomy. […] It is suggested that patients presenting with nipple involvement only and/or small T1 lesions close to the nipple could be treated with wide local excision and axillary dissection in discontinuity followed by radiotherapy to the rest of the breast. […] A total mastectomy without an axillary node dissection is the treatment of choice in this type of patient. […] A therapeutic option of conservative surgical intervention and follow-up is described for five of these select patients, all of whom are presently without evidence of disease with an average follow-up of 50 months.

• #1 Paget’s disease of the breast – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-the-breast/diagnosis-treatment/drc-20351084

  After your operation, your doctor may recommend additional treatment (adjuvant therapy) with anti-cancer drugs (chemotherapy), radiation therapy or hormone therapy to prevent a recurrence of breast cancer. […] Your specific treatment will depend on the extent of the cancer and whether your cancer tests positive for certain characteristics, such as having estrogen or progesterone receptors.

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

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

  These treatments can reduce the risk of the cancer spreading. […] Chemotherapy may help prevent the cancer from returning after treatment, especially if: the tumor was large, the cells had spread to the lymph nodes, the cells are of an aggressive type. […] After treatment finishes, it is essential to attend all follow-up appointments and to continue to monitor for changes. […] If breast cancer is present, the characteristics and stage of the condition will determine the treatment options. These may include surgery, radiation therapy, targeted therapy, and chemotherapy.

• #1 Paget’s Disease of the Breast: Symptoms, Diagnosis, Treatment, and Prevention

  https://www.webmd.com/breast-cancer/pagets-nipple-disease

  The treatment depends on the underlying breast cancer. You might have one or more these: […] Mastectomy. Your surgeon removes your entire breast. […] Lumpectomy. If the disease affects only your nipple and the area around it, your doctor could suggest this treatment to save your breast. […] Radiation. High-energy radiation can kill cancer cells. It might come from a machine outside your body (external) or from a small implant in your breast (internal). […] Chemotherapy. Certain drugs also kill cancer cells. You can get them in a pill that you swallow or in a liquid that goes into your vein (intravenous). […] Hormonal therapy. These drugs keep estrogen from reaching cancer cells so cancer doesn’t grow or come back. They work on cancers that are positive on a hormone receptor (HR) test. You could get a selective estrogen receptor modulator (SERM) or an aromatase inhibitor (AI). They come in pills.

• #1 logo–sylvester

  https://umiamihealth.org/en/sylvester-comprehensive-cancer-center/treatments-and-services/breast-cancer/paget%E2%80%99s-disease-of-the-nipple-

  If you have breast-conserving surgery, rather than a mastectomy, you will also need to have radiation therapy to destroy any cancer cells left behind after surgery. […] Chemotherapy is cancer-fighting medicine that’s administered intravenously or by a pill. Chemotherapy also might be recommended before surgery, called neoadjuvant chemotherapy, to help reduce the size of the tumor and improve surgical results. […] Sylvester offers FDA-cleared cold cap therapy to reduce hair loss during chemotherapy. This therapy works by cooling the scalp during treatment. […] Targeted therapies are treatments designed to attack the molecular changes that make the cancer cells grow and spread. […] After you have surgery, and possibly radiation therapy, you may need to have one or more additional therapies, such as chemotherapy, targeted therapy or hormone therapy. Since most people with Paget’s disease of the nipple also have invasive breast cancer, your treatment will be based on the type and stage of cancer you have.

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

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

  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. Pharmacological treatment includes hormonal therapy and chemotherapy, which may be used depending on the stage and form of the disease. Surgical treatment usually involves mastectomy or segmental resection of the breast. In some cases, adjuvant therapy may be prescribed after surgery to reduce the likelihood of recurrence. […] The following groups of drugs can be used to treat Paget’s disease: hormonal drugs tamoxifen, letrozole; chemotherapy drugs docetaxel, cyclophosphamide; immunotherapy drugs aimed at activating the immune system against the tumor; pain relievers to relieve symptoms and side effects of treatment. […] Treatment includes surgery, chemotherapy and hormone therapy, depending on the patients condition.

• #1 Paget’s Disease of the Breast | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/pagets-disease-of-the-breast

  If the cancer is more invasive, surgery may be combined with other treatments. Other treatments include: Chemotherapy. This treatment uses strong medications to destroy cancer cells. Radiotherapy. This treatment administers controlled doses of radiation to destroy cancer cells. Targeted or hormone therapy. These treatments reduce the risk of the cancer returning and reduce the risk of the cancer spreading. […] If Pagets disease of the breast is caught in its early stages, the prognosis for a full recovery is more likely.

• #1 Paget’s Disease of the Breast (PDB): Diagnosis and Treatment – Los Angeles Times

  https://www.latimes.com/doctors-scientists/medicine/cancer/story/pagets-disease-breast-diagnosis-treatment

  Adjuvant Therapy including Whole Breast Radiation Therapy […] Radiation Therapy: Reduces local recurrence, especially after BCS. Chemotherapy: For invasive cancer or nodal involvement. Endocrine Therapy: For hormone receptor positive tumors, tamoxifen or aromatase inhibitors are used. Targeted Therapy: HER2 positive tumors benefit from trastuzumab and other HER2 directed therapy. […] Emerging therapies like antibody-drug conjugates (e.g. trastuzumab deruxtecan) and immune checkpoint inhibitors are being studied in clinical trials and may add to the treatment options for PDB. Combination therapy targeting both HER2 and PD-1/PD-L1 are showing promising results in early phase trials and may be the future of personalized medicine. […] Multidisciplinary approach is important in the diagnosis and treatment of Pagets disease of the breast. A team of healthcare professionals including breast surgeon, medical oncologist, radiation oncologist and pathologist should work together to develop a treatment plan that is individualized to the patient. Treatment may include surgery, radiation therapy, chemotherapy and hormone therapy depending on the underlying cancer and stage of the disease.

• #1 Effectiveness of photodynamic therapy for mammary and extra-mammary Paget’s disease: a state of the science review | BMC Dermatology | Full Text

  https://bmcdermatol.biomedcentral.com/articles/10.1186/1471-5945-11-13

  Paget’s disease is a rare skin disorder occurring in the breast (mammary) or in the groin, genital, peri-anal and axillary regions (extra-mammary). Typical treatment involves surgical excision, which in the case of extra-mammary Paget’s disease, can lead to significant morbidity. Photodynamic therapy (PDT) which uses a topical or intravenous photosensitizing agent that is activated by a light source to ablate abnormal tissue, offers a minimally invasive alternative. The purpose of this study was to assess the effectiveness of photodynamic therapy in the treatment of Paget’s disease. […] Treatment typically involves surgical excision. However, this can lead to significant morbidity, especially in the case of extra-mammary Paget’s disease, where the lesions are often large. Additionally, the disease primarily affects older populations, not all of whom can tolerate surgery. Thus, given the potential slow progression of the disease, considerable interest in less invasive approaches exists. One such approach is photodynamic therapy (PDT). PDT uses a photosensitizing agent that, when activated by light of a particular wavelength, induces a chemical reaction within the cells, destroying the affected tissue.

• #1 Effectiveness of photodynamic therapy for mammary and extra-mammary Paget’s disease: a state of the science review | BMC Dermatology | Full Text

  https://bmcdermatol.biomedcentral.com/articles/10.1186/1471-5945-11-13

  The purpose of this project was to assess the clinical effectiveness of PDT for mammary and extra-mammary Paget’s disease, based on existing published, peer-reviewed clinical studies. […] Evidence of the effectiveness of PDT for Paget’s disease is promising, but limited. This may, in part, be explained by the rarity of the condition, making controlled comparative clinical trials challenging. […] With the limited amount and low level of evidence available, definitive conclusions on the use of PDT for the treatment of Paget’s disease are not possible. However, given the significant morbidity associated with surgery and the frequency of recurrence associated with alternate treatment options, PDT for primary or recurrent EMPD appears promising and warrants further comparative investigation. In the rare case of MPD without underlying breast cancer, PDT may offer a breast-conserving treatment option or an adjuvant to surgery.

• #1

  https://www.ijcmph.com/index.php/ijcmph/article/view/13517

  Goh SSN, Syn NLX, Lim CJE, Lee RE, Samuel M, Ng CWQ. Oncologic outcomes after breast-conserving surgery with radiotherapy versus mastectomy in patients with Paget’s disease of the breast: systematic review and meta-analysis. Brit J Surg. 2023;110(11):1451-7. […] Bulens P, Vanuytsel L, Rijnders A, van der Schueren E. Breast conserving treatment of Paget’s disease. Radiotherapy Oncol. 1990;17(4):305-9. […] Rzaca M, Tarkowski R. Pagets disease of the nipple treated successfully with cryosurgery: A series of cases report. Cryobiology. 2013;67(1):30-3. […] Tarkowski R, Rzaca M. Cryosurgery in the treatment of women with breast cancer-a review. Gland surgery. 2014;3(2):88-93. […] Yanovsky RL, Bartenstein DW, Rogers GS, Isakoff SJ, Chen ST. Photodynamic therapy for solid tumors: A review of the literature. Photodermatol Photoimmunol Photomed. 2019;35(5):295-303.

• #1 Mammary Paget’s Disease: An Update

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

  Non-surgical or limited surgical approaches have also been utilized to manage MPD. In cases without evidence of underlying disease, non-operative management can offer an effective alternative therapy to traditional breast-conserving therapy. In a study by Bulens, et al., 13 patients with MPD confined to the nipple or surrounding skin without signs of an underlying tumor were treated with radiotherapy alone without surgical resection, with no recurrences detected after 58.6 months of mean follow-up. Alternatively, local excision without radiotherapy may be utilized in MPD cases limited to the skin. Investigational therapies such as photodynamic therapy (PDT) have been used to treat cases of MPD as a less invasive alternative therapy. In PDT, a topical or intravenous photosensitizer drug is administered, and a specific wavelength of light is used to activate the drug, which binds with oxygen to destroy the affected cells. Studies on the non-operative management of PDT are limited, and more research is necessary to determine its safety and effectiveness. The authors have also employed cryoablation of subareolar lesions combined with local excision of the affected nipple and/or areola to manage MPD and carcinoma limited to the skin and subareolar tissue among patients refusing a formal partial mastectomy.

• #1 Mammary Paget’s Disease: Understanding Symptoms, Causes, and Treatment • Yesil Health

  https://yesilhealth.com/your-health/mammary-pagets-disease-understanding-symptoms-causes-and-treatment/

  Emerging treatments, such as immunotherapy, are being explored for their effectiveness in treating various types of breast cancer, including Mammary Pagets Disease. These therapies aim to enhance the bodys immune response against cancer cells. […] Its essential for patients to discuss all available treatment options with their healthcare team to determine the best course of action tailored to their specific situation. Early intervention and a comprehensive treatment plan can significantly improve outcomes for those diagnosed with Mammary Pagets Disease.

• #1

  https://www.ijcmph.com/index.php/ijcmph/article/view/13517

  Yang J, Chen Y, Zhang X, Ziyan T, Shanshan W, Ning Z et al. Immunotherapy may be more appropriate for ERBB2 low-expressing extramammary paget’s disease patients: a prognosis analysis and exploration of targeted therapy and immunotherapy of extramammary paget’s disease patients. Cancer Immunol Immunother. 2024;73(12):252.

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

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

  Paget’s disease of the breast is usually associated with ductal carcinoma in situ (DCIS), limited to the nipple and areola area of the breast. […] Paget’s disease is characterized by an intense lymphocytic response, devoid of the immune-suppressive impact of the PD-L1 pathway, but with occasional CTLA-4 expression. […] Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent. […] Breast-conserving surgery with radiotherapy may be a feasible treatment alternative and sentinel lymph node biopsy should be considered as an appropriate treatment for patients with PD-IDC. […] BCS is a safe alternative to mastectomy, provided a clear surgical margin is achieved and adjuvant radiotherapy used. […] Patients with Paget’s disease are candidates for breast conservation with appropriate preoperative investigations. Oncological outcomes are equivalent to those of mastectomy if surgical margins are achieved and adjuvant radiotherapy is given.

• #1 Paget Disease of the Breast | MD Anderson Cancer Center

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

  Treatment for Paget disease of the breast is similar to treatment for other breast cancers. The main difference occurs during surgery. When a patient undergoes surgery for Paget disease, whether a lumpectomy or mastectomy, the procedure requires the removal of the nipple and areola. […] Because Paget disease is a rare breast cancer, finding a team with the expertise to properly diagnose and treat this cancer is essential. The Nellie B. Connally Breast Center at MD Anderson is one of the largest and most active breast centers in the world, giving our team experience in treating Paget that few can rival. […] Paget disease patients will also benefit from our multidisciplinary approach to care. All MD Anderson breast cancer patients have a breast surgeon, breast medical oncologist and breast radiation oncologist assigned to their case. These experts work together to develop a coordinated, personalized treatment plan for everyone they see. […] MD Anderson patients have access to clinical trials offering promising new treatments that cannot be found anywhere else.

• #1

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

  Targeted therapy: Specific drugs that target characteristics of cancer cells can be effective for certain types of breast cancer. […] Your care team at Advocate Health Care will use a collaborative approach to provide the best treatment possible. We also offer supportive care services such as counseling, nutrition therapy, palliative care and physical therapy that may be beneficial to help you manage the physical and emotional challenges of cancer treatment.

• #1

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

  Targeted therapy: For certain types of breast cancer, drugs that target specific characteristics of cancer cells can be effective. […] Your care team at Aurora Health Care will use a collaborative approach to provide the best treatment possible. Supportive care services such as counseling, nutrition therapy, palliative care and physical therapy may also be offered to help you manage the physical and emotional challenges of cancer treatment.

• #1 Paget’s Disease of the Breast: Stages, Symptoms & Prognosis

  https://my.clevelandclinic.org/health/diseases/17913-pagets-disease-of-the-breast

  If you only have Paget’s disease of the nipple or ductal carcinoma in situ (stage 0) that hasn’t spread beyond the milk ducts, your prognosis is excellent. Most people who discover and treat these conditions early will make a full recovery. […] The earlier you identify and treat it, the better your outcome will be. Many people make a full recovery, but it will require surgery to remove it.

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

• #2

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

  Paget’s disease of the nipple is usually associated with breast cancer. […] It’s treated by removing the cancerous part of the breast, or sometimes the entire breast using a procedure called a mastectomy. […] If you’re diagnosed with Paget’s disease of the nipple, surgery is often the first type of treatment you’ll receive. […] The 2 main types of surgery are: mastectomy surgery to remove the whole breast, which can be followed by reconstructive surgery to recreate the removed breast and breast-conserving surgery where only the cancerous lump (tumour) and a little surrounding breast tissue are removed. […] During a mastectomy, all of your breast tissue, including your nipple, will be removed. […] Breast-conserving surgery aims to save as much of your breast as possible while removing the cancer and a small amount of healthy tissue.

• #2 Practical consensus recommendatons for Paget’s disease in breast cancer

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5909301/

  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. Diagnosis and treatment of Paget’s disease is controversial. Expert oncologists discuss on the update on the approaches of Paget’s disease diagnosis and its treatment options. The surgical treatment of Paget’s disease is controversial. Mastectomy with or without axillary lymph node dissection has long been regarded as the standard therapy for Paget’s disease, even in the absence of other clinical signs of malignancy. The underlying mass is often invasive cancer with a high risk of axillary node metastases. The patients have a poorer prognosis and usually the appropriate therapy is based on the pathologic findings of the mass and axillary staging. In the literature, almost all patients treated with local excision, radiotherapy, or both had the clinical presentation of Paget’s disease without a palpable mass. Dixon et al. reported a high recurrence rate of 40% in patients treated with local excision alone, whose mammograms suggested in situ changes confined to the nipple area. Polgr et al. reported a local recurrence rate of 33% with a median follow-up time of 6 years in 33 patients treated with local excision alone with or without underlying ductal carcinoma in situ confined to the nippleareola complex. They concluded that the local excision alone was not an appropriate treatment for patients with Paget’s disease of the nipple.

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

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

  The aim is to analyse the clinical presentation, treatment and outcomes in patients with Pagets disease with invasive ductal carcinoma (PD-IDC), with special emphasis on the role of surgical treatment. […] Traditional treatment for PD-IDC has been mastectomy due to the common occurrence of sonographically and mammographically hidden multifocal and multicentric malignancies located in breast tissue far from the nipple. However, with the development of imaging technology, breast conservation surgery (BCS) has been found to be a feasible surgical option with low risk for local recurrence in selected patients. […] In addition to BCS, sentinel lymph node biopsy (SLNB) may be effective in patients with PD; however, its role in PD treatment remains unclear. […] The results demonstrated that PD-IDC appears to alter the association between prognosis and Her2 status. Meanwhile, breast-conserving surgery with radiotherapy may be a feasible treatment alternative and sentinel lymph node biopsy should be considered as an appropriate treatment for patients with PD-IDC.

• #2 logo–sylvester

  https://umiamihealth.org/en/sylvester-comprehensive-cancer-center/treatments-and-services/breast-cancer/paget%E2%80%99s-disease-of-the-nipple-

  If you have an invasive cancer in addition to Paget’s disease, your doctor may recommend removing your entire breast, called a mastectomy, to make sure all cancer cells are removed. If you decide you want reconstructive surgery to restore your breast, you can do so as part of your initial surgery or wait until later to have surgery. […] In this surgery, your surgeon removes your entire breast, but leaves your underarm lymph nodes intact. […] With a modified radical mastectomy, your surgeon removes the nipple, areola and all of the breast tissue without removing the underlying muscle. […] Doctors use this minor surgery to determine if cancer has spread beyond a primary tumor into your lymphatic system. A sentinel lymph node is the first place cancer cells may move to when they begin to spread.

• #2 Practical consensus recommendatons for Paget’s disease in breast cancer

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5909301/

  With the increasing diagnosis of the early breast cancers and the findings from multiple randomized, prospective trials demonstrating that breast conserving therapy is a feasible alternative for patients with disease limited to the central segment of the breast, the reported techniques for conservative management of Paget’s disease vary widely. These include nipple excision alone (partial or complete), central segmentectomy alone, these resections plus radiation and radiation without resection. Recent reports from several studies with long-term follow-up have shown that breast-conserving surgery is equivalent to mastectomy in terms of overall and disease-free survival in patients with breast cancer. If conservative therapy is adopted, the patient should be followed up carefully with regular mammography. Mastectomy should be reserved for the few occasions, when relapse occurs.

• #2

  https://link.springer.com/article/10.1007/s11864-004-0047-x

  Pagets disease of the breast is a relatively rare condition, accounting for 1% to 3% of primary breast cancers. It is associated with an underlying carcinoma, invasive or noninvasive (ductal carcinoma in situ), in most of the cases. Primary treatment is surgical with adjuvant therapy being dictated by the stage and nature of the underlying tumor. Modified radical mastectomy is the standard of care with breast conservation appropriate in a select group of patients with Pagets disease. This select group includes patients that are diagnosed with nipple-areola changes alone without evidence of a palpable mass or mammographic abnormality. In this group of patients, breast conservation offers local recurrence rates comparable to rates in patients with invasive or noninvasive cancers. In patients diagnosed with associated palpable masses or mammographic abnormalities suggestive of cancer, the recurrence rates are higher and mastectomy is warranted.

• #2 Mammary Paget’s Disease: An Update

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

  There are no category 1 data that specifically address the local management of MPD according to the 3.2022 version of the National Comprehensive Cancer Network guidelines (accessed on 23 March 2022). Therefore, surgical management remains the main intervention for MPD based on the management of non-MPD-associated breast cancer. Total or skin-sparing mastectomy with surgical axillary staging with or without breast reconstruction is frequently performed for the treatment of MPD due to the frequency of multicentric or multifocal disease. However, with improvements in imaging and patient selection, breast-conserving therapy has become increasingly more common for patients with unifocal disease limited to the nipple-areolar region. Removal of the nipple-areolar complex is generally achieved with a central lumpectomy for removal of the involved nipple and areola en bloc with underlying disease. Superior esthetic results can be achieved with oncoplastic surgical techniques (e.g., Grisotti mastopexy, Wise-Pattern mammaplasty) combined with a contralateral mammaplasty or mastopexy, if desired, to maintain breast symmetry. For optimal esthetic results, immediate or delayed nipple-areolar reconstruction (e.g., C-V Flap) and/or dermatography (medical tattooing) may be performed to create a symmetrical, color-matched nipple-areolar complex following resection of the nipple and/or areola.

• #2 Practical consensus recommendatons for Paget’s disease in breast cancer

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5909301/

  Use of breast radiation therapy alone for the Paget’s disease of the breast has been reported in limited numbers and with varying results. Current studies suggest that irradiation of the breast tissue to a radical dose may be effective against DCIS. This gives support to the view that selected patients with Paget’s disease of the breast can be treated by radiotherapy as an alternative to radical surgery in selected patients with Paget’s disease confined to the nipple, without clinical or radiological detectable breast tumor. […] Both total mastectomy and breast preserving surgery followed by appropriate adjuvant therapy are acceptable treatment options for carefully selected patients with Paget’s disease. The output of this discussion is to provide the best and effective method of diagnosis of disease and provide best treatment options for patients with Paget’s disease of the breast cancer.

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

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

  In our series, there was no difference between surgical treatment subgroups. […] The previously reported techniques for BCS in patients with PD have varied widely and include nipple excision and central segmentectomy as well as resection plus radiation. There is a place for BCS in selected patients with PD of the breast, especially those with no mass. […] Similarly, these studies supported the use of BCS with radiotherapy as a feasible alternative for patients with PD-IDC. […] Although SLNB may still be considered a controversial treatment in patients with PD, SLNB has become a common approach in patients with breast cancer and appears to be a feasible treatment option. […] In the present study, patients undergoing SLNB had similar survival to those receiving ALND regardless of surgical mode of their breast cancer treatment.

• #2 Treatment options for Paget’s disease of the nipple | Cancer Australia

  https://www.canceraustralia.gov.au/cancer-types/breast-cancer/treatment-options/treatment-options-pagets-disease-nipple

  Removal of the whole breast (mastectomy) and/or removal of one or more lymph nodes from the armpit may be needed for some patients. […] As the nipple is likely to be removed, there are a number of options to recreate a nipple. These include having a nipple tattoo or having a nipple made from your own tissue. […] For women with Paget’s disease of the nipple, radiotherapy is almost always recommended after breast conserving surgery. […] Radiotherapy is sometimes recommended after mastectomy. […] Radiotherapy uses X-rays (controlled doses of radiation) to destroy cancer cells. […] Radiotherapy is usually given once a day, 5 days a week for 3–6 weeks. […] For some women with Paget’s disease of the nipple, radiotherapy may be the only treatment needed after the biopsy used during diagnosis.

• #2 Paget’s disease of the breast | Breast Cancer Now

  https://breastcancernow.org/about-breast-cancer/diagnosis/types-of-breast-cancer/pagets-disease-of-the-breast/

  Treatment aims to remove the area of Pagets disease from your breast and reduce the risk of it coming back or spreading to other parts of the body. […] Surgery is usually the first treatment for Pagets disease of the breast. Its often the only treatment youll need if you have Pagets disease without DCIS or invasive breast cancer. […] If youve been diagnosed with Pagets disease alone, youre most likely to be offered surgery to remove your nipple and areola. This is known as a central excision. […] You may be offered other types of surgery if you have also been diagnosed with DCIS or invasive breast cancer, such as breast-conserving surgery or a mastectomy. […] Depending on the features of your Pagets disease, you may also be offered radiotherapy after your surgery. Radiotherapy uses high-energy x-rays to destroy cancer cells. Its used to reduce the risk of the cancer coming back in the same breast.

• #2 Paget’s Disease

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

  Paget’s Disease of the Nipple is an uncommon breast disease and traditionally was treated with mastectomy. […] There may be a role for conservative surgery followed by radiation as the studies below suggest: […] Breast-conserving therapy is a feasible alternative for patients with Paget disease and a limited extent of underlying DCIS. To achieve good local control, treatment should be comprised of a complete excision of the nipple-areolar complex including the underlying disease, followed by irradiation to the whole breast. […] Breast-conserving therapy involving complete resection of the nipple-areola complex followed by definitive radiotherapy is a viable alternative to mastectomy in the treatment of Paget’s disease. High rates of disease free and cause specific survival, in addition to adequate local control, justify consideration of a conservative approach.

• #2 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

  Doctors treat Paget’s disease of the nipple with surgery and radiation. […] The goal of treatment for Pagets disease is to remove as much of the cancer as possible and to prevent cancer cells from spreading. […] Doctors treat Pagets disease with surgery. They may also recommend other cancer treatments, like radiation. […] Surgery is almost always used to treat Pagets disease. Your doctor may recommend breast-conserving surgery, where they remove your nipple and areola but preserve the rest of your breast. […] If your cancer has spread throughout your breast, your doctor may recommend a mastectomy. This surgery removes your entire breast. […] Doctors often treat Paget’s disease with radiation therapy, which uses high-energy rays to destroy cancer cells. Radiation therapy reduces the odds that cancer will come back.

• #2

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

  After having breast-conserving surgery, it’s likely you’ll need to have radiotherapy to destroy any remaining cancer cells. […] If you have a mastectomy, you may be able to have reconstructive surgery to recreate your breast. […] If you decide not to have breast reconstruction, you can wear a false breast or breast prosthesis, which is available free on the NHS. […] After your surgery, you may need further treatment if you have invasive breast cancer (where the cancerous cells have spread into other tissue in your breast). […] Other types of treatment for breast cancer include: chemotherapy where powerful medicine is used to destroy cancer cells and prevent them dividing and growing, radiotherapy where controlled doses of high-energy radiation, usually X-rays, are used to destroy cancer cells, targeted therapy if your breast cancer is HER2 positive, targeted therapy, usually a medicine called trastuzumab, can be used to treat the cancer by stopping the effects of HER2 and helping your immune system fight off cancer cells, hormone therapy if your breast cancer is hormone-receptor positive, hormone therapy can be used to treat the cancer by lowering the levels of hormones in your body or stopping their effects.

• #2 Paget’s Disease of the Breast | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/pagets-disease-of-the-breast

  If the cancer is more invasive, surgery may be combined with other treatments. Other treatments include: Chemotherapy. This treatment uses strong medications to destroy cancer cells. Radiotherapy. This treatment administers controlled doses of radiation to destroy cancer cells. Targeted or hormone therapy. These treatments reduce the risk of the cancer returning and reduce the risk of the cancer spreading. […] If Pagets disease of the breast is caught in its early stages, the prognosis for a full recovery is more likely.

• #2 Breast Paget’s disease. Symptoms, diagnosis and treatment. Clínica Universidad de Navarra

  https://www.cun.es/en/diseases-treatments/diseases/breast-paget-disease

  The therapeutic approach that is assessed in patients with Paget’s disease in the breast will depend, fundamentally, on whether a breast carcinoma exists in addition to Paget’s disease. […] If there is an absence of breast cancer, if Paget’s disease does not have multiple foci (multicentric) or if the underlying disease is relatively close to the areola-nipple complex, the patient may be a candidate for breast conserving surgery. […] Chemotherapy given before surgery may allow some patients with underlying disease to opt for breast-conserving surgery, avoiding a mastectomy. […] In addition, after breast conserving surgery, various reconstructive techniques can be performed on the breast to achieve favorable aesthetics and a better quality of life for patients. […] For those with an underlying malignancy or multicentric disease, mastectomy remains the standard treatment. […] After surgery, most of these patients receive radiation therapy, administered throughout the breast. If nodal involvement is confirmed when the tissue removed in surgery is analyzed, it may be necessary to also administer chemotherapy.

• #2 logo–sylvester

  https://umiamihealth.org/en/sylvester-comprehensive-cancer-center/treatments-and-services/breast-cancer/paget%E2%80%99s-disease-of-the-nipple-

  If you have breast-conserving surgery, rather than a mastectomy, you will also need to have radiation therapy to destroy any cancer cells left behind after surgery. […] Chemotherapy is cancer-fighting medicine that’s administered intravenously or by a pill. Chemotherapy also might be recommended before surgery, called neoadjuvant chemotherapy, to help reduce the size of the tumor and improve surgical results. […] Sylvester offers FDA-cleared cold cap therapy to reduce hair loss during chemotherapy. This therapy works by cooling the scalp during treatment. […] Targeted therapies are treatments designed to attack the molecular changes that make the cancer cells grow and spread. […] After you have surgery, and possibly radiation therapy, you may need to have one or more additional therapies, such as chemotherapy, targeted therapy or hormone therapy. Since most people with Paget’s disease of the nipple also have invasive breast cancer, your treatment will be based on the type and stage of cancer you have.

• #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. […] The usual treatment of mammary Paget disease is surgical excision. Although mastectomy was routine, especially in males, consideration is now given to breast-conserving surgery (removing the nipple, areola, and underlying breast tissue with a margin of healthy tissue). Sentinel lymph node biopsy assesses node involvement. Axillary lymph node dissection may be required if the breast cancer has spread to lymph nodes. […] Further treatment may include: Radiotherapy, if the primary cancer was large or could not be completely excised, or lymph nodes were positive; Tamoxifen, if oestrogen receptor positive; Trastuzumab (Herceptin), if HER2 positive; Chemotherapy, if the breast cancer is rapidly progressive or advanced.

• #2 Paget Disease of the Breast – NCI

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

  How is Paget disease of the breast treated? […] For many years, mastectomy, with or without the removal of lymph nodes under the arm on the same side of chest (known as axillary lymph node dissection), was regarded as the standard surgery for Paget disease of the breast. […] Studies have shown, however, that breast-conserving surgery that includes removal of the nipple and areola, followed by whole-breast radiation therapy, is a safe option for people with Paget disease of the breast who do not have a palpable lump in their breast and whose mammograms do not reveal a tumor. […] People with Paget disease of the breast who have a breast tumor and are having a mastectomy should be offered sentinel lymph node biopsy to see whether the cancer has spread to the axillary lymph nodes. […] Depending on the stage and other features of the underlying breast tumor (for example, the presence or absence of lymph node involvement, estrogen and progesterone receptors in the tumor cells, and HER2 protein overexpression in the tumor cells), adjuvant therapy, consisting of chemotherapy and/or hormonal therapy, may also be recommended.

• #2

  https://www.ijcmph.com/index.php/ijcmph/article/view/13517

  Yang J, Chen Y, Zhang X, Ziyan T, Shanshan W, Ning Z et al. Immunotherapy may be more appropriate for ERBB2 low-expressing extramammary paget’s disease patients: a prognosis analysis and exploration of targeted therapy and immunotherapy of extramammary paget’s disease patients. Cancer Immunol Immunother. 2024;73(12):252.

• #2

  https://www.ijcmph.com/index.php/ijcmph/article/view/13517

  Pagets disease of the breast, or mammary Pagets disease (MPD), is a rare and often misdiagnosed form of breast cancer, typically presenting as eczematous or ulcerative lesions on the nipple-areola complex. […] Traditionally, surgical excision has been the cornerstone of treatment, with mastectomy serving as the standard approach. However, advances in imaging and surgical techniques have introduced breast-conserving surgery (BCS) and oncoplastic methods as viable alternatives, particularly for cases with localized disease. […] In addition to surgical management, adjuvant therapies, including radiation and systemic treatments, are reviewed to highlight their role in reducing recurrence and enhancing patient survival. Emerging treatments such as cryoablation, photodynamic therapy (PDT), and immunotherapy are discussed for their potential as adjuncts or alternatives to traditional surgery.

• #2 Mammary Paget’s Disease: An Update

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

  Non-surgical or limited surgical approaches have also been utilized to manage MPD. In cases without evidence of underlying disease, non-operative management can offer an effective alternative therapy to traditional breast-conserving therapy. In a study by Bulens, et al., 13 patients with MPD confined to the nipple or surrounding skin without signs of an underlying tumor were treated with radiotherapy alone without surgical resection, with no recurrences detected after 58.6 months of mean follow-up. Alternatively, local excision without radiotherapy may be utilized in MPD cases limited to the skin. Investigational therapies such as photodynamic therapy (PDT) have been used to treat cases of MPD as a less invasive alternative therapy. In PDT, a topical or intravenous photosensitizer drug is administered, and a specific wavelength of light is used to activate the drug, which binds with oxygen to destroy the affected cells. Studies on the non-operative management of PDT are limited, and more research is necessary to determine its safety and effectiveness. The authors have also employed cryoablation of subareolar lesions combined with local excision of the affected nipple and/or areola to manage MPD and carcinoma limited to the skin and subareolar tissue among patients refusing a formal partial mastectomy.

• #2

  https://www.ijcmph.com/index.php/ijcmph/article/view/13517

  Goh SSN, Syn NLX, Lim CJE, Lee RE, Samuel M, Ng CWQ. Oncologic outcomes after breast-conserving surgery with radiotherapy versus mastectomy in patients with Paget’s disease of the breast: systematic review and meta-analysis. Brit J Surg. 2023;110(11):1451-7. […] Bulens P, Vanuytsel L, Rijnders A, van der Schueren E. Breast conserving treatment of Paget’s disease. Radiotherapy Oncol. 1990;17(4):305-9. […] Rzaca M, Tarkowski R. Pagets disease of the nipple treated successfully with cryosurgery: A series of cases report. Cryobiology. 2013;67(1):30-3. […] Tarkowski R, Rzaca M. Cryosurgery in the treatment of women with breast cancer-a review. Gland surgery. 2014;3(2):88-93. […] Yanovsky RL, Bartenstein DW, Rogers GS, Isakoff SJ, Chen ST. Photodynamic therapy for solid tumors: A review of the literature. Photodermatol Photoimmunol Photomed. 2019;35(5):295-303.

• #2 Extramammary Paget’s Disease: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/24266-extramammary-pagets-disease

  Providers usually treat extramammary Pagets disease with surgery, including: […] Mohs surgery: During this procedure, your surgeon removes the affected skin cells layer by layer. This technique helps save the surrounding healthy skin. […] Wide local excision: Your surgeon removes the area of EMPD along with some healthy tissue around it. They then sew together the edges of the wound. […] If you cant have surgery due to poor health or other conditions, your provider may suggest treatments such as: […] Immunotherapy. […] Laser skin resurfacing. […] Photodynamic therapy (PDT). […] Radiation therapy. […] Topical medications used to treat cancer (cytotoxic drugs). […] Any underlying cancers will also need additional treatment.

• #2 Paget’s Disease of the Breast (PDB): Diagnosis and Treatment – Los Angeles Times

  https://www.latimes.com/doctors-scientists/medicine/cancer/story/pagets-disease-breast-diagnosis-treatment

  Adjuvant Therapy including Whole Breast Radiation Therapy […] Radiation Therapy: Reduces local recurrence, especially after BCS. Chemotherapy: For invasive cancer or nodal involvement. Endocrine Therapy: For hormone receptor positive tumors, tamoxifen or aromatase inhibitors are used. Targeted Therapy: HER2 positive tumors benefit from trastuzumab and other HER2 directed therapy. […] Emerging therapies like antibody-drug conjugates (e.g. trastuzumab deruxtecan) and immune checkpoint inhibitors are being studied in clinical trials and may add to the treatment options for PDB. Combination therapy targeting both HER2 and PD-1/PD-L1 are showing promising results in early phase trials and may be the future of personalized medicine. […] Multidisciplinary approach is important in the diagnosis and treatment of Pagets disease of the breast. A team of healthcare professionals including breast surgeon, medical oncologist, radiation oncologist and pathologist should work together to develop a treatment plan that is individualized to the patient. Treatment may include surgery, radiation therapy, chemotherapy and hormone therapy depending on the underlying cancer and stage of the disease.

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

  https://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. 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. […] After surgery, radiation therapy and perhaps chemotherapy, targeted therapy, and/or hormone therapy may be recommended. 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. As in other breast cancer diagnoses and treatment, a consultation with a medical oncologist would be prudent.

• #2

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

  Targeted therapy: For certain types of breast cancer, drugs that target specific characteristics of cancer cells can be effective. […] Your care team at Aurora Health Care will use a collaborative approach to provide the best treatment possible. Supportive care services such as counseling, nutrition therapy, palliative care and physical therapy may also be offered to help you manage the physical and emotional challenges of cancer treatment.

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

• #2 Paget’s Disease of the Breast: Stages, Symptoms & Prognosis

  https://my.clevelandclinic.org/health/diseases/17913-pagets-disease-of-the-breast

  If you only have Paget’s disease of the nipple or ductal carcinoma in situ (stage 0) that hasn’t spread beyond the milk ducts, your prognosis is excellent. Most people who discover and treat these conditions early will make a full recovery. […] The earlier you identify and treat it, the better your outcome will be. Many people make a full recovery, but it will require surgery to remove it.

• #2

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

  Surgery is the main treatment for Paget’s disease of the nipple. Depending on whether the cancer has spread, surgery will either involve removing the whole breast (a mastectomy), or the nipple and areola with the breast tissue underneath them (a central excision). […] If the whole of your breast is removed, breast reconstruction surgery can be used to create a breast shape to match your remaining breast. […] You may also need further treatment if you have invasive breast cancer. This may be a combination of: chemotherapy where powerful medicine is used to destroy cancerous cells, radiotherapy where controlled doses of high-energy radiation are used to destroy cancerous cells, targeted or hormone therapy which lowers the risk of cancer returning in the same breast (and affecting the other breast), and it lowers the risk of cancerous cells spreading elsewhere in the body. […] If Paget’s disease is detected and treated in its early stages, there’s a good chance of a full recovery.

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

• #3 The management of Paget’s disease of the breast – PubMed

  https://pubmed.ncbi.nlm.nih.gov/14990209/

  Paget’s disease of the breast is a relatively rare condition, accounting for 1% to 3% of primary breast cancers. […] Primary treatment is surgical with adjuvant therapy being dictated by the stage and nature of the underlying tumor. Modified radical mastectomy is the standard of care with breast conservation appropriate in a select group of patients with Paget’s disease. […] In this group of patients, breast conservation offers local recurrence rates comparable to rates in patients with invasive or noninvasive cancers. […] In patients diagnosed with associated palpable masses or mammographic abnormalities suggestive of cancer, the recurrence rates are higher and mastectomy is warranted.

• #3 Pagets Disease Breast Treatment – Learn Look Locate

  https://learnlooklocate.com/pagets-disease-breast-treatment/

  Paget’s Disease of the breast can be successfully treated with breast-conserving therapy. […] Surgery is the main treatment for Paget’s disease of the nipple. Depending on whether the cancer has spread, surgery will either involve removing the whole breast (a mastectomy), or the nipple and areola with the breast tissue underneath them (a central excision). Paget’s disease confined to the nipple or areola can also be successfully treated with breast-conserving therapy followed by radiation. […] Researchers have increasingly been using breast-conserving therapy, in which only a portion of the breast is surgically removed, for the treatment of Paget’s disease that does not appear to have spread from the area of the nipple instead of mastectomy. […] The researchers concluded that breast-conserving therapy provides equal outcomes to mastectomy in patients with Paget’s disease of the breast that is confined to the nipple or areola. […] The researchers concluded that breast-conserving therapy is an acceptable treatment option for patients with Paget’s disease of the breast that is confined to the nipple or areola region.

• #3

  https://link.springer.com/article/10.1245/ASO.2005.05.026

  The optimal surgical management of Pagets disease of the breast remains to be defined. Mastectomy has been the standard of care, but several institutions have recently advocated breast-conserving surgery, particularly for patients with minimal disease. […] Ninety-two patients (88.5%) underwent mastectomy, and 12 (11.5%) had a breast-conserving procedure. […] Breast-conserving approaches result in local control and survival rates similar to those achieved with mastectomy.

• #3 Practical consensus recommendatons for Paget’s disease in breast cancer

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5909301/

  Use of breast radiation therapy alone for the Paget’s disease of the breast has been reported in limited numbers and with varying results. Current studies suggest that irradiation of the breast tissue to a radical dose may be effective against DCIS. This gives support to the view that selected patients with Paget’s disease of the breast can be treated by radiotherapy as an alternative to radical surgery in selected patients with Paget’s disease confined to the nipple, without clinical or radiological detectable breast tumor. […] Both total mastectomy and breast preserving surgery followed by appropriate adjuvant therapy are acceptable treatment options for carefully selected patients with Paget’s disease. The output of this discussion is to provide the best and effective method of diagnosis of disease and provide best treatment options for patients with Paget’s disease of the breast cancer.

• #3 Paget’s Disease

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

  The results with this breast conserving management suggest a place for radiotherapy in the treatment of Paget’s disease limited to the nipple. […] Radiotherapy is a valuable alternative to mastectomy. […] In this selected group, radical radiotherapy with small fields localised to the involved skin is an effective alternative to mastectomy. […] It is suggested that patients presenting with nipple involvement only and/or small T1 lesions close to the nipple could be treated with wide local excision and axillary dissection in discontinuity followed by radiotherapy to the rest of the breast. […] A total mastectomy without an axillary node dissection is the treatment of choice in this type of patient. […] A therapeutic option of conservative surgical intervention and follow-up is described for five of these select patients, all of whom are presently without evidence of disease with an average follow-up of 50 months.

• #3 Paget’s Disease of the Breast (PDB): Diagnosis and Treatment – Los Angeles Times

  https://www.latimes.com/doctors-scientists/medicine/cancer/story/pagets-disease-breast-diagnosis-treatment

  Pagets disease of the breast is a rare kind of breast cancer that mostly affects the skin around the nipple. […] Treatment of PDB depends on the extent of disease and presence of underlying cancer. Treatment options are surgery, radiation and systemic therapy. […] Radiation Therapy: Reduces local recurrence, especially after BCS. Whole breast radiation therapy is usually recommended after BCS to make sure any remaining cancer cells are killed and reduce the risk of recurrence. […] Surgical Options […] Breast-Conserving Surgery (BCS): For patients with localized disease and no extensive underlying cancer. Usually with radiation. Outcomes are similar to total mastectomy in selected patients. […] Total Mastectomy (TM): Historically the standard of care. Indicated for extensive disease or cases not suitable for BCS.

• #3 Paget’s disease of the breast | Breast Cancer Now

  https://breastcancernow.org/about-breast-cancer/diagnosis/types-of-breast-cancer/pagets-disease-of-the-breast/

  If you also have DCIS or invasive breast cancer, you may be offered other treatments such as chemotherapy, targeted therapy, hormone (endocrine) therapy, or bisphosphonates. […] Treatments given after surgery are called adjuvant treatments. […] If you have invasive breast cancer in addition to Pagets disease, you may be given some treatments before surgery. This is known as neo-adjuvant or primary treatment.

• #3 logo–sylvester

  https://umiamihealth.org/en/sylvester-comprehensive-cancer-center/treatments-and-services/breast-cancer/paget%E2%80%99s-disease-of-the-nipple-

  If you have breast-conserving surgery, rather than a mastectomy, you will also need to have radiation therapy to destroy any cancer cells left behind after surgery. […] Chemotherapy is cancer-fighting medicine that’s administered intravenously or by a pill. Chemotherapy also might be recommended before surgery, called neoadjuvant chemotherapy, to help reduce the size of the tumor and improve surgical results. […] Sylvester offers FDA-cleared cold cap therapy to reduce hair loss during chemotherapy. This therapy works by cooling the scalp during treatment. […] Targeted therapies are treatments designed to attack the molecular changes that make the cancer cells grow and spread. […] After you have surgery, and possibly radiation therapy, you may need to have one or more additional therapies, such as chemotherapy, targeted therapy or hormone therapy. Since most people with Paget’s disease of the nipple also have invasive breast cancer, your treatment will be based on the type and stage of cancer you have.

• #3 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. […] The usual treatment of mammary Paget disease is surgical excision. Although mastectomy was routine, especially in males, consideration is now given to breast-conserving surgery (removing the nipple, areola, and underlying breast tissue with a margin of healthy tissue). Sentinel lymph node biopsy assesses node involvement. Axillary lymph node dissection may be required if the breast cancer has spread to lymph nodes. […] Further treatment may include: Radiotherapy, if the primary cancer was large or could not be completely excised, or lymph nodes were positive; Tamoxifen, if oestrogen receptor positive; Trastuzumab (Herceptin), if HER2 positive; Chemotherapy, if the breast cancer is rapidly progressive or advanced.

• #3 Paget’s Disease of the Breast (PDB): Diagnosis and Treatment – Los Angeles Times

  https://www.latimes.com/doctors-scientists/medicine/cancer/story/pagets-disease-breast-diagnosis-treatment

  Adjuvant Therapy including Whole Breast Radiation Therapy […] Radiation Therapy: Reduces local recurrence, especially after BCS. Chemotherapy: For invasive cancer or nodal involvement. Endocrine Therapy: For hormone receptor positive tumors, tamoxifen or aromatase inhibitors are used. Targeted Therapy: HER2 positive tumors benefit from trastuzumab and other HER2 directed therapy. […] Emerging therapies like antibody-drug conjugates (e.g. trastuzumab deruxtecan) and immune checkpoint inhibitors are being studied in clinical trials and may add to the treatment options for PDB. Combination therapy targeting both HER2 and PD-1/PD-L1 are showing promising results in early phase trials and may be the future of personalized medicine. […] Multidisciplinary approach is important in the diagnosis and treatment of Pagets disease of the breast. A team of healthcare professionals including breast surgeon, medical oncologist, radiation oncologist and pathologist should work together to develop a treatment plan that is individualized to the patient. Treatment may include surgery, radiation therapy, chemotherapy and hormone therapy depending on the underlying cancer and stage of the disease.

• #3

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

  Surgery is the main treatment for Paget’s disease of the nipple. Depending on whether the cancer has spread, surgery will either involve removing the whole breast (a mastectomy), or the nipple and areola with the breast tissue underneath them (a central excision). […] If the whole of your breast is removed, breast reconstruction surgery can be used to create a breast shape to match your remaining breast. […] You may also need further treatment if you have invasive breast cancer. This may be a combination of: chemotherapy where powerful medicine is used to destroy cancerous cells, radiotherapy where controlled doses of high-energy radiation are used to destroy cancerous cells, targeted or hormone therapy which lowers the risk of cancer returning in the same breast (and affecting the other breast), and it lowers the risk of cancerous cells spreading elsewhere in the body. […] If Paget’s disease is detected and treated in its early stages, there’s a good chance of a full recovery.

• #4 Paget’s Disease of the Breast: Stages, Symptoms & Prognosis

  https://my.clevelandclinic.org/health/diseases/17913-pagets-disease-of-the-breast

  Paget’s disease of the breast is a rare type of breast cancer that develops in the skin of the nipple. The primary treatment is surgery. […] Treatment involves surgery and sometimes additional cancer therapies. […] Any cancer in your breast will need to be surgically removed, but how much tissue is removed will depend on your condition. If you only have Paget’s disease of the breast, and no other tumors, you may have more minimal, breast-conserving surgery. […] Depending on how extensive the cancer is, your surgeon may recommend a mastectomy as well. You may have breast reconstruction surgery afterward if you choose. […] After surgery, you’ll likely have additional, complementary treatments to prevent cancer from recurring. Adjuvant therapies may include radiation therapy, chemotherapy, hormone therapy, and immunotherapy.

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