Paget's disease of the breast surgery
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Preeti Singh, M.B.B.S.[2]
Overview
Overview
Mastectomy is the mainstay of treatment for Paget’s disease of the breast. Patients who do not have a palpable lump are treated with removal of the nipple and areola, followed by whole-breast radiation therapy, whereas patients with associated ductal carcinoma in situ or invasive breast cancer are treated with complete resection of the underlying disease with excision of the nipple–areola complex and radiation therapy of the remaining breast tissue. When lymph nodes are involved, more extensive axillary lymph node surgery may be needed.
Surgery
Surgery
Surgery is the mainstay treatment for Paget’s disease of the breast.The indication for surgery depends on:[1][2][3][4][5][6][7][8][9]
- Presence of palpable breast mass.
- Stage, clinical, radiological and histopathological features of the underlying breast cancer.
- Metastatic involvement of lymph nodes.
- Presence of Invasive breast cancer is and indication for axillary lymph node dissection.
| Treatment | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Nipple or areola changes without palpable breast mass | Nipple or areola changes with palpable breast mass | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Positive mammography or ultrasonography | No clinically or radiographically demonstrable lesion | Cenetral segmentectomy including the nipple-areola complex | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Unifocal disease | Multicentric disease | Breast-conserving therapy with central segmentectomy of the nipple-areolar region postoperative breast irradiation is optional | Multicentric disease | Involved margins | Unifocal disease with negative margins | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Radical mastectomy with sentinel lymph node biopsy | Re-excision or completion radical mastectomy with sentinel lymph node biopsy | Postoperative radiation therapy | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Radical mastectomy of the nipple-areola complex and the suspicious underlying lesion followed by postoperative radiation and sentinel lymph node biopsy | Radical mastectomy with sentinel lymph node biopsy | Mastectomy should be considered when multicentric disease is discovered | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Nipple or areola changes without palpable breast mass or mammographic abnormality
- Mastectomy is considered as the standard therapy for Paget’s disease of the breast.
- However, breast-conserving surgery that includes removal of the nipple areola complex and lumpectomy, followed by whole-breast or moderate-dose radiation therapy to eradicate micro-invasion, is a safe option for people with Paget’s disease of the breast who do not have a palpable lump in their breast and whose mammograms do not reveal a tumor.
- Paget’s disease associated with ductal carcinoma in situ observation without radiation therapy can be considered in patients with negative margins.
- Immunohistochemical stains for hormone receptor (Estrogen or progesterone receptor) and HER2 receptor should be done as they effect prognosis and treatment.
- Breast reconstruction should be considered for cosmetic appearance.
Nipple or areola changes with palpable breast mass or mammographic abnormality
- Paget’s disease associated with invasive breast cancer treatment should include complete resection of the underlying disease with excision of the nipple–areola complex and radiation therapy of the remaining breast tissue.
- Sentinel lymph node biopsy should be offered to all patients to see whether the cancer has spread to the axillary lymph nodes.
- If cancer cells are histologically found in the sentinel lymph node(s), more extensive axillary lymph node surgery is performed depending on the extent.
- Immunohistochemical stains for hormone receptor (Estrogen or progesterone receptor) and HER2 receptor should be done for adjuvent treatment.
- Reconstruction of breast as well as nipple areolar complex should be considered for cosmetic appearance.
References
References
- ↑ Duan X, Sneige N, Gullett AE, Prieto VG, Resetkova E, Andino LM, Wu Y, Gilcrease MZ, Bedrosian I, Dawood S, Arun B, Albarracin CT (September 2012). “Invasive paget disease of the breast: clinicopathologic study of an underrecognized entity in the breast”. Am. J. Surg. Pathol. 36 (9): 1353–8. doi:10.1097/PAS.0b013e318259ef7f. PMID 22895267.
- ↑ Wong SM, Freedman RA, Sagara Y, Stamell EF, Desantis SD, Barry WT, Golshan M (December 2015). “The effect of Paget disease on axillary lymph node metastases and survival in invasive ductal carcinoma”. Cancer. 121 (24): 4333–40. doi:10.1002/cncr.29687. PMID 26376021.
- ↑ Gaspari, Eleonora; Ricci, Aurora; Liberto, Valeria; Scarano, Angela Lia; Fornari, Maria; Simonetti, Giovanni (2013). “An Unusual Case of Mammary Paget’s Disease Diagnosed Using Dynamic Contrast-Enhanced MRI”. Case Reports in Radiology. 2013: 1–5. doi:10.1155/2013/206235. ISSN 2090-6862.
- ↑ Song Q, Jin Y, Huang T, Zhang JH (2015). “Diagnosis and treatment of Paget’s disease of the breast: an analysis of 72 cases”. Int J Clin Exp Med. 8 (10): 19616–20. PMC 4694522. PMID 26770622.
- ↑ Bijker N, Rutgers EJ, Duchateau L, Peterse JL, Julien JP, Cataliotti L (February 2001). “Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients”. Cancer. 91 (3): 472–7. PMID 11169928.
- ↑ Adams SJ, Kanthan R (October 2016). “Paget’s disease of the male breast in the 21st century: A systematic review”. Breast. 29: 14–23. doi:10.1016/j.breast.2016.06.015. PMID 27394005.
- ↑ Piekarski J, Jeziorski A, Baklinska M, Szymczak W, Zadrozny M, Berner J (March 2004). “Patients with Paget disease of nipple and with palpable mass in breast have unfavorable prognosis”. J. Exp. Clin. Cancer Res. 23 (1): 33–7. PMID 15149148.
- ↑ Wu Q, Ding X, Li J, Sun S, Zhu S, Wu J, Liu Q, Yao F, Sun S (April 2017). “Surgical treatment in Paget’s disease with invasive ductal carcinoma: an observational study based on SEER”. Sci Rep. 7: 45510. doi:10.1038/srep45510. PMC 5395813. PMID 28422090.
- ↑ Stockdale AD, Brierley JD, White WF, Folkes A, Rostom AY (September 1989). “Radiotherapy for Paget’s disease of the nipple: a conservative alternative”. Lancet. 2 (8664): 664–6. PMID 2570909.
Looking for the patient version?
© 2026 MyEClinic – IFTM Institut für Telematik in der Medizin GmbH