Paget’s disease of the breast – observational retrospective study of the past 20 years
Authors:
L. Gabrielová 1; V. Hanincová 1; O. Coufal 1,2; M. Holánek 3,4; O. Zapletal 1
Authors‘ workplace:
Klinika operační onkologie, Oddělení mamokutánní a onkoplastické chirurgie, Masarykův onkologický ústav, Brno
1; Klinika operační onkologie, Lékařská fakulta Masarykovy univerzity, Brno
2; Klinika komplexní onkologické péče, Masarykův onkologický ústav, Brno
3; Klinika komplexní onkologické péče, Lékařská fakulta Masarykovy univerzity, Brno
4
Published in:
Rozhl. Chir., 2021, roč. 100, č. 4, s. 173-179.
Category:
Original articles
doi:
https://doi.org/10.33699/PIS.2021.100.4.
Overview
Introduction: Paget‘s disease of the breast (PD) is a rare type of carcinoma that affects the skin of the nipple-areolar complex. Unresolved issues exist regarding its diagnosis and therapy. The aim of the study was to gather data on how the therapy of the disease is approached in clinical practice, and to formulate current diagnostic and therapeutic recommendations.
Methods: Retrospective evaluation of data from medical records of patients with PD who underwent surgery at our department between 2001 and 2020. The data was evaluated using basic statistical methods.
Results: Sixty four female patients with the mean age of 62.5 years. In 58 women, PD was confirmed before surgery, with the median of 20 weeks from initial symptoms to diagnosis. Forty seven of the patients were operated for presumed isolated PD; in 38 cases, histopathological evaluation of the specimen revealed an associated malignancy in the mammary gland. Primary breast-conserving surgery (BCS) was performed in 46 patients; surgical revision was indicated in 17 cases. In 6 patients with PD associated with non-invasive breast cancer treated by BCS without radiotherapy (RT), a local recurrence appeared in 3 cases, which is significantly more compared to the group of patients undergoing total mastectomies (p=0.032). No local recurrence appeared in 9 cases of isolated PD treated by BCS, including 6 patients without RT. The tumors associated with PD were mostly ER-negative (44/57) and HER2-positive (22/25).
Conclusion: In cases where PD is suspected, careful clinical examination and the use of available diagnostic imaging techniques including MRI are appropriate. BCS without RT is not an adequate oncological therapy where an associated malignancy of the breast is found.
Keywords:
Paget‘s disease of the breast − non-invasive breast cancer − invasive breast cancer − breast-conserving surgery − total mastectomy
Sources
- Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St. Bartholowmew Hospital Reports 1874;10:87−89.
- Yao Y, Sun L, Meng Y, et al. Breast-conserving surgery in patients with mammary Paget’s disease. J Surg Res. 2019;241:178−187. doi: 10.1016/j.jss.2019.03.025.
- Kawase K, Dimaio DJ, Tucker SL, et al. Paget’s disease of the breast: there is a role for breast-conserving therapy. Ann Surg Oncol. 2005 May;12(5):391−397. doi: 10.1245/ASO.2005.05.026. Epub 2005 Mar 29.
- Adams SJ, Kanthan R. Paget’s disease of the male breast in the 21st century: A systematic review. Breast 2016 Oct;29:14−23. doi: 10.1016/j.breast.2016.06.015. Epub 2016 Jul 6.
- Chen S, Chen H, Yi Y, et al. Comparative study of breast cancer with or without concomitant Paget disease: An analysis of the SEER database. Cancer Med. 2019 Jul;8(8):4043−4054. doi: 10.1002/cam4.2242. Epub 2019 May 28.
- Ling H, Hu X, Xu XL, et al. Patients with nipple-areola Paget‘s disease and underlying invasive breast carcinoma have very poor survival: a matched cohort study. PLoS One. 2013 Apr 19;8(4):e61455. doi: 10.1371/journal.pone.0061455.
- Streng A, Gutjahr E, Aulmann S, et al. Pathologie der Mamillenregion: I. Morbus Paget der Mamille, Varianten und Differenzialdiagnosen. Pathologe 2020 Jul;41(4):393−399. doi: 10.1007/s00292-020-00772-1. PMID: 32405655.
- Sandoval-Leon AC, Drews-Elger K, Gomez-Fernandez CR, et al. Paget’s disease of the nipple. Breast Cancer Res Treat. 2013 Aug;141(1):1−12. doi: 10.1007/s10549-013-2661-4. Epub 2013 Aug 9.
- Arafah M, Arain SA, Raddaoui EMS, et al. Molecular subtyping of mammary Paget’s disease using immunohistochemistry. Saudi Med J. 2019 May;40(5):440−446. doi: 10.15537/smj.2019.5.23967.
- Drews-Elger K, Sandoval-Leon AC, Ergonul AB, et al. Paget’s disease of the nipple in a Her2-positive breast cancer xenograft model. Breast Cancer Res Treat. 2020;179:577–584. doi: 10.1007/s10549-019-05490-8.
- Lloyd J, Flanagan AM. Mammary and extramammary Paget’s disease. J Clin Pathol. 2000 Oct;53(10):742−749. doi: 10.1136/jcp.53.10.742.
- Wong SM, Freedman RA, Stamell E, et al. Modern trends in the surgical management of Paget’s disease. Ann Surg Oncol. 2015 Oct;22(10):3308−3316. doi: 10.1245/s10434-015-4664-3. Epub 2015 Jul 23.
- Gaurav A, Gupta V, Koul R, et al. Practical consensus recommendations for Paget’s disease in breast cancer. South Asian J Cancer 2018 Apr−Jun;7(2):83−86. doi: 10.4103/sajc.sajc_107_18.
- Yasir M, Lotfollahzadeh S. Mammary Paget disease. In: StatPearls [Internet]. Treasure Island (FL): Stat Pearls Publishing 2020.
- Sakorafas GH, Blanchard DK, Sarr MG, et al. Paget’s disease of the breast: a clinical perspective. Langenbecks Arch Surg. 2001 Nov;386(6):444−450. doi: 10.1007/s004230100250. Epub 2001 Oct 17.
- Helme S, Harvey K, Agrawal A. Breast-conserving surgery in patients with Paget’s disease. Br J Surg. 2015 Sep;102(10):1167−1174. doi: 10.1002/bjs.9863. Epub 2015 Jul 14.
- Moon JY, Chang YW, Lee EH, et al. Malignant invasion of the nipple-areolar complex of the breast: usefulness of breast MRI. AJR Am J Roentgenol. 2013 Aug;201(2):448−455. doi: 10.2214/AJR.12.9186.
- Lim HS, Jeong SJ, Lee JS, et al. Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation. Radiographics 2011 Nov-Dec;31(7):1973−1987. doi: 10.1148/rg.317115070.
- Gao Y, Brachtel EF, Hernandez O, et al. An analysis of nipple enhancement at breast MRI with radiologic-pathologic correlation. Radiographics 2019 Jan-Feb;39(1):10−27. doi: 10.1148/rg.2019180039. Epub 2018 Dec 14.
- TNM Klasifikace zhoubných novotvarů, 8. vydání (originál 2017) Region: ČR, rok: 2017. ISBN: 978-80-7472-173-1.
- Farouk O, Attia E, Roshdy S, et al. The outcome of oncoplastic techniques in defect reconstruction after resection of central breast tumors. World J Surg Oncol. 2015 Sep 26;13:285. doi: 10.1186/s12957-015-0688-1.
- Siponen E, Hukkinen K, Heikkilä P, et al. Surgical treatment in Paget’s disease of the breast. Am J Surg. 2010 Aug;200(2):241−246. doi: 10.1016/j.amjsurg.2009.07.044.
- Marshall JK, Griffith KA, Haffty BG, et al. Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer 2003 May 1;97(9):2142−2149. doi: 10.1002/cncr.11337.
- Nicholson BT, Harvey JA, Cohen MA. Nipple-areolar complex: normal anatomy and benign and malignant processes. Radiographics 2009 Mar−Apr;29(2):509−523. doi: 10.1148/rg.292085128.
- Polgár C, Orosz Z, Kovács T, et al. Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients. Cancer 2002 Mar 15;94(6):1904−1905. doi: 10.1002/cncr.10405.
- Dixon AR, Galea MH, Ellis IO, et al. Paget‘s disease of the nipple. Br J Surg. 1991 Jun;78(6):722−723. doi: 10.1002/bjs.1800780627.
- Dalberg K, Hellborg H, Wärnberg F. Paget’s disease of the nipple in a population based cohort. Breast Cancer Res Treat. 2008 Sep;111(2):313−319. doi: 10.1007/s10549-007-9783-5. Epub 2007 Oct 19.
- Pierce LJ, Haffty BG, Solin LJ, et al. The conservative management of Paget‘s disease of the breast with radiotherapy. Cancer. 1997 Sep 15;80(6):1065−1072.
- Dominici LS, Lester S, Liao GS, et al. Current surgical approach to Paget‘s disease. Am J Surg. 2012 Jul;204(1):18−22. doi: 10.1016/j.amjsurg.2011.07.010. Epub 2011 Oct 28.
- Fourquet A, Campana F, Vielh P, et al. Paget‘s disease of the nipple without detectable breast tumor: conservative management with radiation therapy. Int J Radiat Oncol Biol Phys. 1987 Oct;13(10):1463−1465. doi: 10.1016/0360-3016(87)90311-7.
- Li YJ, Huang XE, Zhou XD. Local breast cancer recurrence after mastectomy and breast-conserving surgery for Paget’s disease: A meta-analysis. Breast Care ( Basel) 2014 Dec;9(6):431−434. doi: 10.1159/000368431.
- Coufal O, Selingerová I, Vrtělová P, et al. A simple model to assess the probability of invasion in ductal carcinoma in situ of the breast diagnosed by needle biopsy. Biomed Res Int. 2014;2014:480840. doi: 10.1155/2014/480840. Epub 2014 Jul 8.
- Vrtělová P, Coufal O, Pavlík T, et al. Viditelnost na ultrasonografii jako nejsilnější prediktor invazivity u duktálních karcinomů in situ v retrospektivní studii. Klin Onkol. 2009;22(6):278−283.
Labels
Surgery Orthopaedics Trauma surgeryArticle was published in
Perspectives in Surgery
2021 Issue 4
Most read in this issue
- Paget’s disease of the breast – observational retrospective study of the past 20 years
- Granulomatous mastitis − treatment options and our experience
- Breast cancer − medical oncology summary 2021
- Ductal carcinoma in situ and the extent of surgery