Paget's disease of the breast surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Preeti Singh, M.B.B.S.[2]

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 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]


 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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


Nipple or areola changes with palpable breast mass or mammographic abnormality

References

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.


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