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Paget disease of the nipple

MedGen UID:
352830
•Concept ID:
C1704323
•
Neoplastic Process
Synonyms: Mammary Paget disease; Nipple Paget Disease; Nipple Paget's Disease; nipple Paget's disease; Paget disease of nipple; Paget disease of the breast; Paget Disease of the Nipple; Paget's carcinoma of the nipple; Paget's disease of nipple; Paget's Disease of Nipple; Paget's Disease of the Breast; Paget's Disease of the Nipple; Paget's disease of the nipple; Paget's disease, mammary; PD
SNOMED CT: Paget's disease of nipple (403946000); Paget disease of nipple (403946000); Paget's carcinoma of the nipple (2985005)
 
Monarch Initiative: MONDO:0015873
Orphanet: ORPHA180275

Definition

Paget disease of the nipple describes a rare presentation of breast cancer, seen most frequently in women aged 50-60, manifesting with nipple drainage and itching, erythema, crusty and excoriated nipple, thickened plaques, and hyperpigmentation (less frequently). It is due to tumor cells invading the nipple-areola complex and represents 1-3% of all new breast cancer diagnoses. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPaget disease of the nipple

Professional guidelines

PubMed

Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients.
Polgár C, Orosz Z, Kovács T, Fodor J
Cancer 2002 Mar 15;94(6):1904-5. doi: 10.1002/cncr.10405. PMID: 11920556

Recent clinical studies

Etiology

Multimodality Imaging of Benign and Malignant Diseases of the Nipple-Areolar Complex.
Guirguis MS, Arribas EM, Kapoor MM, Patel MM, Perez F, Nia ES, Ding Q, Moseley TW, Adrada BE
Radiographics 2024 Apr;44(4):e230113. doi: 10.1148/rg.230113. PMID: 38483829
Treatment of Mammary Paget Disease: A systematic review and meta-analysis of real-world data.
Lin CW, Chiang MH, Tam KW
Int J Surg 2022 Nov;107:106964. Epub 2022 Oct 26 doi: 10.1016/j.ijsu.2022.106964. PMID: 36309195
Dermatoses of the nipple.
Parlakgumus A, Yildirim S, Bolat FA, Caliskan K, Ezer A, Colakoglu T, Moray G
Can J Surg 2009 Apr;52(2):160-1. PMID: 19399214Free PMC Article
Paget's disease of the nipple.
Fine RM
Int J Dermatol 1986 Jun;25(5):298-9. doi: 10.1111/j.1365-4362.1986.tb02247.x. PMID: 3013788
Paget's disease of the nipple.
Kay S
Surg Gynecol Obstet 1966 Nov;123(5):1010-4. PMID: 4288767

Diagnosis

Paget's disease of the breast: diagnosis and management.
Hudson-Phillips S, Cox K, Patel P, Al Sarakbi W
Br J Hosp Med (Lond) 2023 Jan 2;84(1):1-8. Epub 2023 Jan 24 doi: 10.12968/hmed.2022.0439. PMID: 36708338
Nipple eczema: A systematic review and practical recommendations.
Reynaert V, Gutermuth J, Wollenberg A
J Eur Acad Dermatol Venereol 2023 Jun;37(6):1149-1159. Epub 2023 Feb 16 doi: 10.1111/jdv.18920. PMID: 36695082
Cutaneous Squamous Cell Carcinoma of the Nipple Presenting as Paget's Disease.
Rebielak M, Wolf M, Oxenberg J
Am Surg 2022 Mar;88(3):521-522. Epub 2020 Oct 22 doi: 10.1177/0003134820945233. PMID: 33090013
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Dermatoses of the nipple.
Parlakgumus A, Yildirim S, Bolat FA, Caliskan K, Ezer A, Colakoglu T, Moray G
Can J Surg 2009 Apr;52(2):160-1. PMID: 19399214Free PMC Article

Therapy

Changes following nipple areolar complex reconstruction and tattooing resembling a recurrent Paget's disease of the breast.
Hanafiah M, Alhabshi SM, Mahin AH
BMJ Case Rep 2013 Oct 28;2013 doi: 10.1136/bcr-2013-201281. PMID: 24165504Free PMC Article
Paget's disease of the nipple.
Sandoval-Leon AC, Drews-Elger K, Gomez-Fernandez CR, Yepes MM, Lippman ME
Breast Cancer Res Treat 2013 Aug;141(1):1-12. Epub 2013 Aug 9 doi: 10.1007/s10549-013-2661-4. PMID: 23929251
Dermatoses of the nipple.
Parlakgumus A, Yildirim S, Bolat FA, Caliskan K, Ezer A, Colakoglu T, Moray G
Can J Surg 2009 Apr;52(2):160-1. PMID: 19399214Free PMC Article
Dermatologic diseases of the breast in young women.
Ward KA, Burton JL
Clin Dermatol 1997 Jan-Feb;15(1):45-52. doi: 10.1016/s0738-081x(96)00109-5. PMID: 9034655
Florid papillomatosis of male nipple after diethylstilbestrol therapy.
Waldo ED, Sidhu GS, Hu AW
Arch Pathol 1975 Jul;99(7):364-6. PMID: 167700

Prognosis

Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Paget's disease of the nipple.
Sandoval-Leon AC, Drews-Elger K, Gomez-Fernandez CR, Yepes MM, Lippman ME
Breast Cancer Res Treat 2013 Aug;141(1):1-12. Epub 2013 Aug 9 doi: 10.1007/s10549-013-2661-4. PMID: 23929251
Paget's disease of the nipple.
Fine RM
Int J Dermatol 1986 Jun;25(5):298-9. doi: 10.1111/j.1365-4362.1986.tb02247.x. PMID: 3013788
Significance of eczema in Paget's disease of the breast.
Maor M
Eur J Cancer (1965) 1979 Jan;15(1):35-8. doi: 10.1016/0014-2964(79)90202-0. PMID: 217690
Paget's disease of the nipple.
Kay S
Surg Gynecol Obstet 1966 Nov;123(5):1010-4. PMID: 4288767

Clinical prediction guides

Treatment of Mammary Paget Disease: A systematic review and meta-analysis of real-world data.
Lin CW, Chiang MH, Tam KW
Int J Surg 2022 Nov;107:106964. Epub 2022 Oct 26 doi: 10.1016/j.ijsu.2022.106964. PMID: 36309195
Clinical and sonographic features of nipple lesions.
Cai S, Wang H, Zhu Q, Li J, Sun Q, Jiang Y
Medicine (Baltimore) 2020 Apr;99(15):e19728. doi: 10.1097/MD.0000000000019728. PMID: 32282731Free PMC Article
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Diseases of the male nipple and areola.
Krause W
J Dtsch Dermatol Ges 2011 Dec;9(12):1004-9. Epub 2011 Jun 9 doi: 10.1111/j.1610-0387.2011.07720.x. PMID: 21651722
Some observations on Paget's disease of the nipple.
TOKER C
Cancer 1961 Jul-Aug;14:653-72. doi: 10.1002/1097-0142(199007/08)14:4<653::aid-cncr2820140402>3.0.co;2-v. PMID: 13777220

Recent systematic reviews

Nipple eczema: A systematic review and practical recommendations.
Reynaert V, Gutermuth J, Wollenberg A
J Eur Acad Dermatol Venereol 2023 Jun;37(6):1149-1159. Epub 2023 Feb 16 doi: 10.1111/jdv.18920. PMID: 36695082
Treatment of Mammary Paget Disease: A systematic review and meta-analysis of real-world data.
Lin CW, Chiang MH, Tam KW
Int J Surg 2022 Nov;107:106964. Epub 2022 Oct 26 doi: 10.1016/j.ijsu.2022.106964. PMID: 36309195
Paget's disease of the male breast in the 21st century: A systematic review.
Adams SJ, Kanthan R
Breast 2016 Oct;29:14-23. Epub 2016 Jul 6 doi: 10.1016/j.breast.2016.06.015. PMID: 27394005

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