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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

Recent clinical studies

Etiology

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
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
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
Adams SJ, Kanthan R
Breast 2016 Oct;29:14-23. Epub 2016 Jul 6 doi: 10.1016/j.breast.2016.06.015. PMID: 27394005
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

Diagnosis

O'Meara S, Cullen IM
Int J STD AIDS 2023 Sep;34(10):735-739. Epub 2023 Apr 25 doi: 10.1177/09564624231171196. PMID: 37097071
Gilmore R, Prasath V, Habibi M
Adv Exp Med Biol 2020;1252:133-136. doi: 10.1007/978-3-030-41596-9_18. PMID: 32816273
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
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
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

Therapy

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
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
Lohsiriwat V, Martella S, Rietjens M, Botteri E, Rotmensz N, Mastropasqua MG, Garusi C, De Lorenzi F, Manconi A, Sommario M, Barbieri B, Cassilha M, Minotti I, Petit JY
Ann Surg Oncol 2012 Jun;19(6):1850-5. Epub 2012 Feb 10 doi: 10.1245/s10434-012-2226-5. PMID: 22322949
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
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

Prognosis

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
Plutino FM, Del Medico P, Vescio G, Fava MG
Ann Ital Chir 2022 May 5;11 PMID: 35588206
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
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
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

Clinical prediction guides

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
Samreen N, Madsen LB, Chacko C, Heller SL
Br J Radiol 2021 Apr 1;94(1120):20201013. Epub 2021 Feb 5 doi: 10.1259/bjr.20201013. PMID: 33544650Free PMC Article
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
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
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

Recent systematic reviews

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
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
Adams SJ, Kanthan R
Breast 2016 Oct;29:14-23. Epub 2016 Jul 6 doi: 10.1016/j.breast.2016.06.015. PMID: 27394005

Supplemental Content

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