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Mammary Paget disease

MedGen UID:
18263
•Concept ID:
C0030185
•
Neoplastic Process
Synonyms: Paget disease of the breast; Paget's disease of the nipple; Paget's disease, mammary
SNOMED CT: Mammary Paget's disease (2985005); Paget disease, mammary (2985005); Paget's disease, mammary (2985005); Paget's disease of breast (2985005)
 
Monarch Initiative: MONDO:0002648

Definition

A malignant neoplasm in which there is infiltration of the skin overlying the breast by neoplastic large cells with abundant pale cytoplasm and large nuclei with prominent nucleoli (Paget cells). It is almost always associated with an intraductal or invasive ductal carcinoma of the breast. The clinical features include focal skin reddening, and eczema. Retraction of the nipple may sometimes occur. [from NCI]

Professional guidelines

PubMed

Spectrum of Changes in Anogenital Mammary-like Glands in Primary Extramammary (Anogenital) Paget Disease and Their Possible Role in the Pathogenesis of the Disease.
Konstantinova AM, Spagnolo DV, Stewart CJR, Kacerovska D, Shelekhova KV, Plaza JA, Suster S, Bouda J, Kyrpychova L, Michal M, Belousova IE, Kerl K, Kazakov DV
Am J Surg Pathol 2017 Aug;41(8):1053-1058. doi: 10.1097/PAS.0000000000000857. PMID: 28614205

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Breast Cancer, 2023

Breast Cancer, NCCN Guidelines Version 4.2022

Recent clinical studies

Etiology

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
CK 8∕18: the key to differentiating intracutaneous lesions with pagetoid features.
Tinca AC, Cocuz IG, Şincu MC, Niculescu R, Sabău AH, Chiorean DM, Morariu SH, Turdean SG, Cotoi OS
Rom J Morphol Embryol 2021 Jul-Sep;62(3):757-763. doi: 10.47162/RJME.62.3.13. PMID: 35263404Free PMC Article
Expression of lipoma preferred partner in mammary and extramammary Paget disease.
Liu Y, Wang Y, Qi R, Mao X, Jin F
Medicine (Baltimore) 2020 Dec 18;99(51):e23443. doi: 10.1097/MD.0000000000023443. PMID: 33371071Free PMC Article
Paget disease of the vulva an analysis of 24 cases.
Loiacono RMR, Traversi P, Deliso MA, Gargano G, Kardhashi A, Francescato R, Loizzi V, Spinelli M, Lovascio S, Fiorito M, Resta L, Popescu O, Cicinelli E, Cormio G
Medicine (Baltimore) 2019 Oct;98(41):e17018. doi: 10.1097/MD.0000000000017018. PMID: 31593076Free PMC Article
Expression of CD3, PD-L1 and CTLA-4 in mammary and extra-mammary Paget disease.
Karpathiou G, Chauleur C, Hathroubi S, Habougit C, Peoc'h M
Cancer Immunol Immunother 2018 Aug;67(8):1297-1303. Epub 2018 Jun 25 doi: 10.1007/s00262-018-2189-x. PMID: 29943071

Diagnosis

Immunohistochemical expression of TRPS1 in mammary Paget disease, extramammary Paget disease, and their close histopathologic mimics.
Cho WC, Ding Q, Wang WL, Nagarajan P, Curry JL, Torres-Cabala CA, Ivan D, Albarracin CT, Sahin A, Prieto VG, Aung PP
J Cutan Pathol 2023 May;50(5):434-440. Epub 2023 Mar 1 doi: 10.1111/cup.14414. PMID: 36808637
Expression of lipoma preferred partner in mammary and extramammary Paget disease.
Liu Y, Wang Y, Qi R, Mao X, Jin F
Medicine (Baltimore) 2020 Dec 18;99(51):e23443. doi: 10.1097/MD.0000000000023443. PMID: 33371071Free PMC Article
Paget disease of the vulva an analysis of 24 cases.
Loiacono RMR, Traversi P, Deliso MA, Gargano G, Kardhashi A, Francescato R, Loizzi V, Spinelli M, Lovascio S, Fiorito M, Resta L, Popescu O, Cicinelli E, Cormio G
Medicine (Baltimore) 2019 Oct;98(41):e17018. doi: 10.1097/MD.0000000000017018. PMID: 31593076Free PMC Article
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Mammary Paget disease.
Burke MJ, Middleton TO
J Am Osteopath Assoc 2013 Sep;113(9):712. doi: 10.7556/jaoa.2013.038. PMID: 24005092

Therapy

Phase II Study of Afatinib in Patients With Tumors With Human Epidermal Growth Factor Receptor 2-Activating Mutations: Results From the National Cancer Institute-Molecular Analysis for Therapy Choice ECOG-ACRIN Trial (EAY131) Subprotocol EAY131-B.
Bedard PL, Li S, Wisinski KB, Yang ES, Limaye SA, Mitchell EP, Zwiebel JA, Moscow JA, Gray RJ, Wang V, McShane LM, Rubinstein LV, Patton DR, Williams PM, Hamilton SR, Conley BA, Arteaga CL, Harris LN, O'Dwyer PJ, Chen AP, Flaherty KT
JCO Precis Oncol 2022 Jul;6:e2200165. doi: 10.1200/PO.22.00165. PMID: 35939768Free PMC Article
Toker cell hyperplasia in Zuska disease: A tricky association.
Torre-Castro J, Haya-Martínez L, Ruffin-Vicente B, Moya-Martínez C, Núñez-Hipólito L, Díaz de la Pinta J, Cullen-Aravena D, Jo-Velasco M, Requena L
J Cutan Pathol 2021 Jan;48(1):180-183. Epub 2020 Nov 10 doi: 10.1111/cup.13908. PMID: 33128407
Treatment of recurrent vulvar Paget disease with imiquimod cream: a case report and review of the literature.
Tonguc E, Güngor T, Var T, Ozat M, Sahin I, Sirvan L
Arch Gynecol Obstet 2011 Jan;283(1):97-101. Epub 2010 Aug 29 doi: 10.1007/s00404-010-1653-4. PMID: 20803209
Complete resolution of Paget disease of the vulva with imiquimod cream.
Hatch KD, Davis JR
J Low Genit Tract Dis 2008 Apr;12(2):90-4. doi: 10.1097/LGT.0b013e31815a58a5. PMID: 18369301

Prognosis

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
CK 8∕18: the key to differentiating intracutaneous lesions with pagetoid features.
Tinca AC, Cocuz IG, Şincu MC, Niculescu R, Sabău AH, Chiorean DM, Morariu SH, Turdean SG, Cotoi OS
Rom J Morphol Embryol 2021 Jul-Sep;62(3):757-763. doi: 10.47162/RJME.62.3.13. PMID: 35263404Free PMC Article
A strengthening the reporting of observational studies in epidemiology (STROBE): Are HE4 and CA 125 suitable to detect a Paget disease of the vulva?
Dellino M, Gargano G, Tinelli R, Carriero C, Minoia C, Tetania S, Silvestris E, Loizzi V, Paradiso A, Casamassima P, Tufaro A, Cormio G, Garrisi VM
Medicine (Baltimore) 2021 Feb 5;100(5):e24485. doi: 10.1097/MD.0000000000024485. PMID: 33592901Free PMC Article
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Expression of CD3, PD-L1 and CTLA-4 in mammary and extra-mammary Paget disease.
Karpathiou G, Chauleur C, Hathroubi S, Habougit C, Peoc'h M
Cancer Immunol Immunother 2018 Aug;67(8):1297-1303. Epub 2018 Jun 25 doi: 10.1007/s00262-018-2189-x. PMID: 29943071

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
Expression of lipoma preferred partner in mammary and extramammary Paget disease.
Liu Y, Wang Y, Qi R, Mao X, Jin F
Medicine (Baltimore) 2020 Dec 18;99(51):e23443. doi: 10.1097/MD.0000000000023443. PMID: 33371071Free PMC Article
Angiogenesis in mammary Paget disease: histopathological analyses of blood vessel density and angiogenic factors.
Akishima-Fukasawa Y, Honma N, Ogata H, Akasaka Y, Mikami T
Diagn Pathol 2020 Jun 11;15(1):75. doi: 10.1186/s13000-020-00988-y. PMID: 32527320Free PMC Article
Paget disease of the vulva an analysis of 24 cases.
Loiacono RMR, Traversi P, Deliso MA, Gargano G, Kardhashi A, Francescato R, Loizzi V, Spinelli M, Lovascio S, Fiorito M, Resta L, Popescu O, Cicinelli E, Cormio G
Medicine (Baltimore) 2019 Oct;98(41):e17018. doi: 10.1097/MD.0000000000017018. PMID: 31593076Free PMC Article
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904

Recent systematic reviews

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

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Breast Cancer, 2023
    • NCCN, 2023
      Breast Cancer, NCCN Guidelines Version 4.2022

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