Paget’s disease of the breast (a review of literature)
https://doi.org/10.17650/1994-4098-2016-12-4-37-46
Abstract
Paget’s disease (PD) of the nipple is a rare form of breast cancer, characterized by erythema, an scaling of the skin and eczematous changes of the nipple-areolar complex. Despite a significant increase in newly detected cases of breast cancer, PD is accounting for 0.5–5.0 % of all breast tumors in the world. In female patients with PD ages range from 24 to 90 years (average age range 50–60 years), predominantly menopausal age in 70 % of cases. But cases of PD in young person were reported.
Currently, the relevance of the PD of the nipple is preserved. There are different methods of treatment, but we haven’t specific international recommendations for treatment PD in surgery, irradiation and chemotherapy or target therapy. There are small numbers of randomized studies of PD. The use of modern methods of instrumental and molecular diagnostics can help designed algorithm the treatment of PD.
In this literature review the etiology, clinical features and current approaches to diagnosis and possible treatment of PD of the nipple.
About the Authors
E. A. Nikitina
N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
M. I. Nechushkin
N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
A. V. Trigolosov
N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
Ya. V. Vishnevskaya
N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
Russian Federation
23 Kashirskoe Shosse, Moscow 115478
References
1. Velpeau S. A treatise on diseases of the breast and mammary region. London: Sydenham Society, 1856. P. 3.
2. Valdes E.K., Feldman S.M. Paget’s disease of the breast. Breast J 2006;12(1):83. PMID: 16409595.
3. Погодина Е.М. Рак Педжета молочной железы: клиника, диагностика, лечение Вестник РОНЦ им. Н.Н. Блохина 1995;6(2):32–7. [Pogodina E.M. Paget’s disease of the breast: clinical aspects, diagnostics and treatment. Vestnik RONC im. N.N. Blokhina = Bulletin of the N.N. Blokhin RCRC 1995;6(2):32–7. (In Russ.)].
4. Баженова А.П., Островцев Л.Д., Хаханашвили Г.Н. Рак молочной железы. М., 1985. [Bazhenova A.P., Ostrovtsev L.D., Khakhanashvili G.N. Breast cancer. Moscow, 1985. (In Russ.)].
5. Поспелов А.И. О болезни Педжета. Протоколы Московского венерологического и дерматологического общества 1894;(4):89. [Pospelov A.I. About Paget’s disease. Protokoly Moskovskogo venerologicheskogo i dermatologicheskogo obshchestva = Reports of the Moscow Venerological and Dermatological Society 1894;(4):89. (In Russ.)].
6. Трапезников Н.Н., Летягин В.П., Алиев Д.А. Лечение опухолей молочной железы. М., 1989. [Trapeznikov N.N., Letyagin V.P., Aliev D.A. Treatment of breast tumors. Moscow, 1989. (In Russ.)].
7. Karakas C. Paget’s disease of the breast. J Carcinog 2011;10:31. DOI: 10.4103/1477-3163.90676. PMID: 22279416.
8. Lim H.S., Jeong S.J., Lee J.S. et al. Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation. Radiographics 2011;31(7):1973–87. DOI: 10.1148/rg.317115070. PMID: 22084182.
9. Sek P., Zawrocki A., Biernat W., Piekarski J.H. HER2 molecular subtype is a dominant subtype of mammary Paget’s cells. An immunohistochemical study. Histopathology 2010;57(4):564–71. DOI: 10.1111/j.1365-2559.2010.03665.x. PMID: 20955381.
10. Lohsiriwat V., Martella S., Rietjens M. et al. Paget’s disease as a local recurrence after nipple-sparing mastectomy: clinical presentation, treatment, outcome, and risk factor analysis. Ann Surg Oncol 2012;19(6):1850–5. DOI: 10.1245/s10434-012-2226-5. PMID: 22322949.
11. Meibodi N.T., Ghoyunlu V.M., Javidi Z., Nahidi Y. Clinicopathologic evaluation of mammary Paget’s disease. Indian J Dermatol 2008;53(1):21–3. DOI: 10.4103/0019-5154.39736. PMID: 19967013.
12. Martin V.G., Pellettiere E.V., Gress D., Miller A.W. Paget’s disease in an adolescent arising in a supernumerary nipple. J Cutan Pathol 1994;21(3):283–6. PMID: 7962832.
13. Muir R. Pathogenesis of Paget’s disease of the nipple and associated lesions. Br J Surg 1935;22:728–37.
14. Inglis K. Paget’s disease of the nipple: with special reference to changes in the ducts. Am J Pathol 1946;22(1):1–33. PMID: 19970857.
15. Yim J.H., Wick M.R., Philpott G.W. et al. Underlying pathology in mammary Paget’s Disease. Ann Surg Oncol 1997;4(4):287–92. PMID: 9181226.
16. Elston C.A., Elston D.M. Mammary Paget disease. Medscape reference. Drugs, diseases and Procedures. 2015.
17. Погодина Е.М., Высоцкая И.В., Сосновских И.А., Зикиряходжаев А.Д. Рак Педжета молочной железы. Маммология 2006;(1):65–70. [Pogodina E.M., Vysotskaya I.V., Sosnovskikh I.A., Zikiryakhodzhayev A.D. Paget’s carcinoma of the breast. Mammologiya = Mammology 2006;(1):65–70. (In Russ.)].
18. Marucci G., Betts C.M., Golouh R. et al. Toker cells are probably precursors of Paget cell carcinoma: a morphological and ultrastructural description. Virchows Arch 2002;441(2):117–
19. DOI: 10.1007/s00428-001-0581-x. PMID: 12189500.
20. Toker C. Clear cells of the nipple epidermis. Cancer 1970;25(3):601–10. PMID: 4313654.
21. Kuan S.F., Montag A.G., Hart J. et al. Differential expression of mucin genes in mammary and extramammary Paget’s disease. Am J Surg Pathol 2001;25(12):1469–77. PMID: 11717535.
22. Morandi L., Pession A., Marucci G.L. et al. Intraepidermal cells of Paget’s carcinoma of the breast can be genetically different from those of the underlying carcinoma. Hum Pathol 2003;34(12):1321–30. PMID: 14691919.
23. Marczyk E., Kruczak A., Ambicka A. et al. The routine immunohistochemical evaluation in Paget disease of the nipple. Pol J Pathol 2011;62(4):229–35. PMID: 22246908.
24. Liegl B., Horn L.C., Moinfar F. Androgen receptors are frequently expressed in mammary and extramammary Paget’s disease. Mod Pathol 2005;18(10):1283–8. DOI: 10.1038/modpathol.3800437. PMID: 15920545.
25. Ellis P.E., Fong L.F., Rolfe K.J. et al. The role of p53 and Ki67 in Paget’s disease of the vulva and the breast. Gynecol Oncol 2002;86(2):150–6. PMID: 12144821.
26. Schelfhout V.R., Coene E.D., Delaey B. et al. Pathogenesis of Paget’s disease: epidermal heregulin-alpha, motility factor, and the HER receptor family. J Natl Cancer Inst 2000;92(8):622–8. PMID: 10772679.
27. Ellis P.E., Cano S.D., Fear M. et al. Reduced E-cadherin expression correlates with disease progression in Paget’s disease of the vulva but not Paget’s disease of the breast. Mod Pathol 2008;21(10):1192–9. DOI: 10.1038/modpathol.2008.50. PMID: 18469796.
28. Kanitakis J. Mammary and extramammary Paget’s disease. J Eur Acad Dermatol Venereol 2007;21(5):581–90. PMID: 17447970.
29. Kanwar A.J., De D., Vaiphei K. et al. Extensive mammary Paget’s disease. Clin Exp Dermatol 2007;32(3):326–7. DOI: 10.1111/j.1365-2230.2006.02326.x. PMID: 17397359.
30. Ucar A.E., Korukluoglu B., Ergul E. et al. Bilateral Paget disease of the male nipple: first report. Breast 2008;17(3):317–8. DOI: 10.1016/j.breast.2007.11.007. PMID: 18171616.
31. Fu W., Mittel V.K., Young S.C. Paget disease of the breast: analysis of 41 patients. Am J Clin Oncol 2001;24(4):397–400. PMID: 11474272.
32. Newell M.S., Birdwell R.L., D’Orsi C.J. et al. ACR Appropriateness Criteria® on nonpalpable mammographic findings (excluding calcifications). J Am Coll Radiol 2010;7(12):920–30. DOI: 10.1016/j.jacr.2010.07.006. PMID: 21129682.
33. Sahoo S., Green I., Rose P.P. Bilateral Paget disease of the nipple associated with lobular carcinoma in situ. Arch Pathol Lab Med 2002;126(1):90–2. DOI: 10.1043/0003-9985(2002)126<0090:BPDOTN>2.0.CO;2. PMID: 11800657.
34. Chen C.Y., Sun L.M., Anderson B.O. Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S. Cancer 2006;107(7):1448–58. DOI: 10.1002/cncr.22137. PMID: 16933329.
35. Dominici L.S., Lester S., Liao G.S. et al. Current surgical approach to Paget’s disease. Am J Surg 2012;204(1):18–22. DOI: 10.1016/j.amjsurg.2011.07.010. PMID: 22036205.
36. Wong S.M., Freedman R.A., Stamell E. et al. Modern trends in the surgical management of Paget’s disease. Ann Surg Oncol 2015;22(10):3308–16. DOI: 10.1245/s10434-015-4664-3. PMID: 26202552.
37. Kao G.F., Graham J.H., Helwig E.B. Paget’s disease of the ectopic breast with an underlying intraductal carcinoma: report of a case. J Cutan Pathol 1986;13(1):59–66. PMID: 3009569.
38. Sakorafas G.H., Blanchard K., Sarr M.G., Farley D.R. Paget’s disease of the breast. Cancer Treat Rev 2001;27(1):9–18. PMID: 11237774.
39. Kollmorgen D.R., Varanasi J.S., Edge S.B., Carson W.E. 3rd. Paget’s disease of the breast: a 33-year experience. J Am Coll Surg 1998;187(2):171–7. PMID: 9704964.
40. Kothari A.S., Beechey-Newman N., Hamed H. et al. Paget disease of the nipple: a multifocal manifestation of higher-risk disease. Cancer 2002;95(1):1–7. DOI: 10.1002/cncr.10638. PMID: 12115309.
41. Barnes P.J., Dumont R.J., Higgins H.G. Acinar pattern of mammary Paget’s disease: a case report. Breast J 2007;13(5):520–6. DOI: 10.1111/j.1524-4741.2007.00477.x. PMID: 17760678.
42. Mobini N. Acantholytic anaplastic Paget’s disease. J Cutan Pathol 2009;36(3):374–80. DOI: 10.1111/j.1600-0560.2008.01053.x. PMID: 19220635.
43. Mrhalova M., Kodet R. Paget’s disease of the nipple: a copy number of the genes ERBB2 and CCND1 versus expression of the proteins ERBB-2 and cyclin D1. Neoplasma 2009;50(6):396–402. PMID: 14689059.
44. Oiso N., Kawara S., Inui H., Kawada A. Pigmented spots as a sign of mammary Paget’s disease. Clin Exp Dermatol 2009;34(1):36–8. DOI: 10.1111/j.1365-2230.2008.02876.x. PMID: 18627392.
45. Sandoval-Leon A.C., Drews-Elger K., Gomez-Fernandez C.R. et al. Paget’s disease of the nipple. Breast Cancer Res Treat 2013;141(1):1–12. DOI: 10.1007/s10549-013-2661-4. PMID: 23929251.
46. Nicholson B.T., Harvey J.A., Cohen M.A. Nipple-areolar complex: normal anatomy and benign and malignant processes. Radiographics 2009;29(2):509–23. DOI: 10.1148/rg.292085128. PMID: 19325062.
47. Morrogh M., Morris E.A., Liberman L. et al. MRI identifies otherwise occult disease in select patients with Paget disease of the nipple. J Am Coll Surg 2008;206(2):316–21. DOI: 10.1016/j.jamcollsurg.2007.07.046. PMID: 18222386.
48. Kim H.S., Seok J.H., Cha E.S. et al. Significance of nipple enhancement of Paget’s disease in contrast enhanced breast MRI. Arch Gynecol Obstet 2010;282(2):157–62. DOI: 10.1007/s00404-009-1244-4. PMID: 19838723.
49. Veronesi U., Cascinelli N., Mariani L. et al. Twenty-year follow-up of a randomized study comparing breast-conserving surgery with radical mastectomy for early breast cancer. N Engl J Med 2002;347(16):1227–32. DOI: 10.1056/NEJMoa020989. PMID: 12393819.
50. Polgár C., Orosz Z., Kovács T., Fodor J. 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;94(6): 1904–5. PMID: 11920556.
51. Dixon A.R., Galea M.H., Ellis I.O. et al. Paget’s disease of the nipple. Br J Surg 1991;78(6):722–3. PMID: 1648987.
52. Ashikari R., Park K., Huvos A.G., Urban J.A. Paget’s disease of the breast. Cancer 1970;26(3):680–5. PMID: 4318756.
53. Chaudary M.A., Millis R.R., Lane E.B., Miller N.A. Paget’s disease of the nipple: a ten-year review including clinical, pathological, and immunohistochemical findings. Breast Cancer Res Treat 1986;8(2):139–46. PMID: 2434164.
54. Dalberg K., Hellborg H., Warnberg F. Paget’s disease of the nipple in a population based cohort. Breast Cancer Res Treat 2008;111(2):313–9. DOI: 10.1007/s10549-007-9783-5. PMID: 17952590.
55. Singh A., Sutton R.J., Baker C.B., Sacks N.P. Is mastectomy overtreatment for Paget’s disease of the nipple? Breast 1999;8(4):191–4. DOI: 10.1054/brst.1999.0025. PMID: 14731439.
56. Onoe S., Kinoshita T., Tamura N. et al. Feasibility of breast conserving surgery for Paget’s disease. Breast 2011;20(6):515–8. DOI: 10.1016/j.breast.2011.05.010. PMID: 21689938.
57. 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;13(10):1463–5. PMID: 3040645.
58. Marshall J.K., Griffith K.A., Haffty B.G. et al. Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer 2003;97(9):2142–9. DOI: 10.1002/cncr.11337. PMID: 12712465.
59. Trebska-McGowan K., Terracina K.P., Takabe K. Update on the surgical management of Paget’s disease. Gland Surg 2013;2(3):137–42. DOI: 10.3978/j.issn.2227-684X.2013.08.03. PMID: 24386631.
60. Siponen E., Hukkinen K., Heikkila P. et al. Surgical treatment in Paget’s disease of the breast. Am J Surg 2010;200(2):241–6. DOI: 10.1016/j.amjsurg.2009.07.044. PMID: 20678619.
61. Caliskan M., Gatti G., Sosnovskikh I. et al. Paget’s disease of the breast: the experience of the European Institute of Oncology and review of the literature. Breast Cancer Res Treat 2008;112(3):513–21. DOI: 10.1007/s10549-007-9880-5. PMID: 18240020.
62. Иванов В.Г., Жильцова Е.К., Иванова О.А. и др. О влиянии характера хирургического лечения на частоту встречаемости рака Педжета соска. Злокачественные опухоли 2015; (4-2):157–8. [Ivanov V.G., Zhil’tsova E.K., Ivanova O.A. et al. Effect of surgical treatment type on the frequency of Paget’s disease of the nipple. Zlokachestvennye opukholi = Malignant Tumors 2015;(4-2):157–8. (In Russ.)].
63. Ling H., Hu X., Xu X.L. 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;8(4):e61455. DOI: 10.1371/journal.pone.0061455. PMID: 23620755.
64. Стенина М.Б., Владимирова Л.Ю., Гладков О.А. и др. Практические рекомендации по лекарственному лечению рака молочной железы. Злокачественные опухоли 2015;(4 спецвыпуск):99–115. [Stenina M.B., Vladimirova L.Yu., Gladkov O.A. et al. Practical recommendations for breast cancer pharmacotherapy. Zlokachestvennye opukholi = Malignant Tumors 2015;(4 Spec Issue):99–115. (In Russ.)].
65. Senkus E., Kyriakides S., Ohno S. et al. Primary breast cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and followup. Ann Oncol 2015;26(Suppl 5):v8–30. DOI: 10.1093/annonc/mdv298. PMID: 26314782.
66. NCCN Guidelines Version 2.2015. Breast cancer updates. Special situations: Paget’s disease. 2015. URL: http://www.nccn.org/professionals/physician_gls/pdf/breast.pdf (last access date 25.12.2016).
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For citations:
Nikitina E.A., Nechushkin M.I., Trigolosov A.V., Vishnevskaya Ya.V. Paget’s disease of the breast (a review of literature). Tumors of female reproductive system. 2016;12(4):37-46. (In Russ.) https://doi.org/10.17650/1994-4098-2016-12-4-37-46
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