Breast Cancer Research and Treatment

, Volume 111, Issue 2, pp 313–319 | Cite as

Paget’s disease of the nipple in a population based cohort

  • Kristina Dalberg
  • Henrik Hellborg
  • Fredrik Wärnberg
Epidemiology

Abstract

Background Paget’s disease of the nipple is a rare form of breast cancer characterised by the presence of intraepidermal tumour cells. It is often associated with ductal carcinoma in situ (DCIS) and/or invasive cancer in the breast parenchyma. We have studied the presentation and symptoms of Paget’s disease, local control and breast cancer corrected survival following breast conserving surgery or mastectomy. Patients and methods The study is based on 223 women with histological verified Paget’s disease of the nipple diagnosed between 1976 and 2001 at 13 Swedish hospitals. All women´s charts were reviewed. All recurrences and deaths were registered. A comparison was made for differences in breast cancer-corrected survival (BCS) and disease-free survival (DFS) in univariate analyses. Results The median follow-up was 12 (4–28) years. In a vast majority (98%), the main presenting symptom was eczema or ulceration of the nipple. The diagnosis of an underlying breast malignancy was established in 79% of the women before surgery. A cone excision of the nipple-areola complex was performed in 43 women and 169 women had a mastectomy. Eleven elderly women were not operated. One hundred and seventeen women had a non-invasive Paget of which 40 had an underlying DCIS. Invasive cancer was seen in 68 women. In 38 cases the histopathological report did not state if the tumour was invasive or not. Thirty-three women died from breast cancer. In operated women BCS and DFS at 10 years were 87% and 82%, respectively. The 10-year BCS for non-operated patients (n = 11) was 34%. At 10 years, the cumulative local recurrence rate was 9%, 8% among women undergoing mastectomy and 16% among those treated with breast conserving surgery. In univariate analysis the type of surgery, cone excision or mastectomy, had no statistically significant impact on BCS or DFS. Risk factors for breast cancer death and recurrence were having an underlying invasive cancer compared with an in situ carcinoma and having a palpable tumour in the breast. Conclusion The main presenting symptoms were eczema or ulceration of the nipple. Patients with non invasive Pagets disease of the nipple had an excellent cancer outcome. Selected patients with Paget’s disease of the nipple were treated with breast conserving surgery with survival rates similar to those achieved with mastectomy.

Keywords

Paget’s disease of the nipple Symtoms Treatment Prognosis 

References

  1. 1.
    WHO classification (1982) Am J Clin Pathology 78:806–816Google Scholar
  2. 2.
    National Board of health and welfare (2003) Cancer incidence in Sweden 2003. Available: http:// www.socialstyrelsen.se/NR/rdonlyres/86E6C7D0-4650-43DA-82DF-FAC11CDB54C5/3019720044210.pdf
  3. 3.
    van Dongen JA, Voogd AC, Fentiman IS, et al (2000) Long-term results of a randomized trial comparing breast-conserving therapy with mastectomy: European Organization for Research and Treatment of Cancer 10801 trial. J Natl Cancer Inst 92:1143–1150PubMedCrossRefGoogle Scholar
  4. 4.
    Veronesi U, Cascinelli N, Mariani L, et al (2002) Twenty-year follow-up of a randomized study comparing breast-conserving surgery with radical mastectomy for early breast cancer. N Engl J Med 347:1227–1232PubMedCrossRefGoogle Scholar
  5. 5.
    Fisher B, Anderson S, Bryant J, et al (2002) Twenty-year follow up of a randomized trial comparing total mastectomy, lumpectomy, and lumpectomy plus irradiation for the treatment of invasive breast cancer. N Engl J Med 347:1233–1241PubMedCrossRefGoogle Scholar
  6. 6.
    Poggi MM, Danforth DN, Sciuto LC, et al (2003) Eighteen-year results in the treatment of early breast carcinoma with mastectomy versus breast conservation therapy. The National Cancer Institute Randomized Trial. Cancer 98:697–702PubMedCrossRefGoogle Scholar
  7. 7.
    Kawase K, Dimaio DJ, Tucker SL, Buchholz TA, Ross MI, Feig BW, Kuerer HM, Meric-Bernstam F, Babiera G, Ames FC, Singletary SE, Hunt KK (2005) Paget’s disease of the breast: there is a role for breast-conserving therapy. Ann Surg Oncol 12(5):391–397. Epub 2005 Mar 29PubMedCrossRefGoogle Scholar
  8. 8.
    Marshall JK, Griffith KA, Haffty BG, Solin LJ, Vicini FA, McCormick B, Wazer DE, Recht A, Pierce LJ (2003) Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer 97(9):2142–2149PubMedCrossRefGoogle Scholar
  9. 9.
    Polgar C, Orosz Z, Kovacs T, Fodor J (2002) Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer Study of 61 patients. Cancer 94:1904–1905PubMedCrossRefGoogle Scholar
  10. 10.
    Dixon AR, Galea MH, Ellis IO, Elston CW, Blamey RW (1991) Paget’s disease of the nipple. Br J Surg 78:722–723PubMedCrossRefGoogle Scholar
  11. 11.
    Bijker N, Rutgers EJ, Duchateau L, Peterse JL, Julien JP, Cataliotti L (2001) EORTC Breast Cancer Cooperative GroupBreast-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):472–477PubMedCrossRefGoogle Scholar
  12. 12.
    Fourquet A, Campana F, Vielh P, Schlienger P, Jullien D, Vilcoq JR (1987) Paget’s disease of the nipple without detectable breast tumour: conservative management with radiation therapy. Int J Radiat Oncol Biol Phys 13:1463–1465PubMedGoogle Scholar
  13. 13.
    Jamali FR, Ricci A Jr, Deckers PJ (1996) Paget’s disease of the nipple-areola complex. Surg Clin North Am 76(2):365–381PubMedCrossRefGoogle Scholar
  14. 14.
    Kothari AS, Beechey-Newman N, Hamed H, Fentiman IS, D’Arrigo C, Hanby AM, Ryder K (2002) Paget disease of the nipple: a multifocal manifestation of higher-risk disease. Cancer 95(1):1–7PubMedCrossRefGoogle Scholar
  15. 15.
    Eaker S, Dickman PW, Bergkvist L, Holmberg L (2006) Uppsala/Orebro Breast Cancer Group. Differences in management of older women influence breast cancer survival: results from a population-based database in Sweden. PLoS Med 3(3):e25PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC. 2007

Authors and Affiliations

  • Kristina Dalberg
    • 1
  • Henrik Hellborg
    • 2
  • Fredrik Wärnberg
    • 3
  1. 1.Division of Surgery, Department of Clinical ScienceDanderyd Hospital, Karolinska InstitutetStockholmSweden
  2. 2.Oncologic CentreKarolinska University HospitalStockholmSweden
  3. 3.Department of SurgeryUppsala University HospitalUppsalaSweden

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