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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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPaget disease of the nipple

Neoplasm
- Epithelial neoplasm
  - Carcinoma
    - Adenocarcinoma
      - Breast adenocarcinoma
        Mammary Paget disease
        Paget 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

See all (1)

Recent clinical studies

Etiology

Florid papillomatosis of the nipple.

Dinesh BJ, Hayati F, Azizan N, Abdul Rashid NF
BMJ Case Rep 2019 Sep 18;12(9) doi: 10.1136/bcr-2019-231516. PMID: 31537599 Free PMC Article

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

The management of Paget's disease of the breast.

Marcus E
Curr Treat Options Oncol 2004 Apr;5(2):153-60. doi: 10.1007/s11864-004-0047-x. PMID: 14990209

Paget's disease of the breast.

Sakorafas GH, Blanchard K, Sarr MG, Farley DR
Cancer Treat Rev 2001 Feb;27(1):9-18. doi: 10.1053/ctrv.2000.0203. PMID: 11237774

Breast cancer.

Berg JW, Hutter RV
Cancer 1995 Jan 1;75(1 Suppl):257-69. doi: 10.1002/1097-0142(19950101)75:1+<257::aid-cncr2820751311>3.0.co;2-y. PMID: 8001000

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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

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 breast.

Marques-Costa JC, Cuzzi T, Carneiro S, Parish LC, Ramos-e-Silva M
Skinmed 2012 May-Jun;10(3):160-5; quiz 165. PMID: 22779098

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Therapy

Paget's disease of the male breast: case report and a point of view from actual literature.

Vergine M, Musella A, Gulotta E, Frusone F, De Luca A, Maceli F, Libia A, Benedetti Panici P, Monti M
G Chir 2018 Mar-Apr;39(2):114-117. PMID: 29694313

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 treated successfully with cryosurgery: a series of cases report.

Rzaca M, Tarkowski R
Cryobiology 2013 Aug;67(1):30-3. Epub 2013 May 7 doi: 10.1016/j.cryobiol.2013.04.006. PMID: 23665146

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: 19399214 Free PMC Article

Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients.

Bijker N, Rutgers EJ, Duchateau L, Peterse JL, Julien JP, Cataliotti L; EORTC Breast Cancer Cooperative Group
Cancer 2001 Feb 1;91(3):472-7. doi: 10.1002/1097-0142(20010201)91:3<472::aid-cncr1024>3.0.co;2-q. PMID: 11169928

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Prognosis

Extramammary Paget disease.

St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904

Skin diseases of the breast and nipple: Benign and malignant tumors.

Waldman RA, Finch J, Grant-Kels JM, Stevenson C, Whitaker-Worth D
J Am Acad Dermatol 2019 Jun;80(6):1467-1481. Epub 2018 Nov 16 doi: 10.1016/j.jaad.2018.08.066. PMID: 30452954

Mammary and extramammary Paget's disease.

Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N
An Bras Dermatol 2015 Mar-Apr;90(2):225-31. doi: 10.1590/abd1806-4841.20153189. PMID: 25830993 Free 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

Mammary and extramammary Paget's disease.

Kanitakis J
J Eur Acad Dermatol Venereol 2007 May;21(5):581-90. doi: 10.1111/j.1468-3083.2007.02154.x. PMID: 17447970

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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: 32282731 Free PMC Article

Extramammary Paget disease.

St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904

The routine immunohistochemical evaluation in Paget disease of the nipple.

Marczyk E, Kruczak A, Ambicka A, Mularz K, Harazin-Lechowska A, Moskal J, Sokołowski A, Mituś J, Ryś J
Pol J Pathol 2011 Dec;62(4):229-35. PMID: 22246908

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

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