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Extramammary Paget disease

MedGen UID:
45280
•Concept ID:
C0030186
•
Neoplastic Process
Synonyms: Extra Mammary Paget Disease; Extra Mammary Paget's Disease; Extra-Mammary Paget Disease; Extra-Mammary Paget's Disease; Extra-Mammary Pagets Disease; Extramammary Paget Disease; Extramammary Paget's Disease; Extramammary Pagets Disease; Extramammary, Paget Disease; Paget Disease Extramammary; Paget Disease, Extra Mammary; Paget Disease, Extra-Mammary; Paget Disease, Extramammary; Paget's Disease, Extra Mammary; Paget's Disease, Extra-Mammary; Paget's Disease, Extramammary; Pagets Disease, Extra-Mammary; Pagets Disease, Extramammary
SNOMED CT: Paget's disease, extramammary (except Paget's disease of bone) (71447003); Paget disease, extramammary (except Paget disease of bone) (71447003); Paget disease of skin (232336001); Paget's disease of skin (232336001)
 
Monarch Initiative: MONDO:0008177
OMIM®: 167300
Orphanet: ORPHA2800

Definition

A rare skin tumor characterized by predominantly intraepithelial growth of an adenocarcinoma which may either arise primarily in the skin (primary extramammary Paget disease) or result from intraepithelial spread of a visceral carcinoma (secondary extramammary Paget disease). The lesion is typically located in the anogenital region, presenting as a scaly, oozing, pruritic or painful erythematous plaque often resembling eczema. It may exhibit an invasive component with a significant risk of lymph node metastasis. [from ORDO]

Clinical features

From HPO
Neoplasm
MedGen UID:
10294
•Concept ID:
C0027651
•
Neoplastic Process
An organ or organ-system abnormality that consists of uncontrolled autonomous cell-proliferation which can occur in any part of the body as a benign or malignant neoplasm (tumor).
See: Feature record | Search on this feature
Eczematoid dermatitis
MedGen UID:
3968
•Concept ID:
C0013595
•
Disease or Syndrome
Eczema is a form of dermatitis that is characterized by scaly, pruritic, erythematous lesions located on flexural surfaces.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtramammary Paget disease
Follow this link to review classifications for Extramammary Paget disease in Orphanet.

Professional guidelines

PubMed

Treatment of high tumor mutation burden metastatic extramammary Paget disease with an anti-PD-1 antibody.
Nakayama Y, Ogata D, Wada S, Tsuruta S, Matsui Y, Okumura M, Hiki K, Nakano E, Namikawa K, Yamazaki N
J Dermatol 2023 Nov;50(11):e396-e397. Epub 2023 Jul 17 doi: 10.1111/1346-8138.16898. PMID: 37458156
Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.
Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, Aasi SZ, Aung PP, Barker CA, Bolotin D, Bordeaux JS, Cartee TV, Chandra S, Cho NL, Choi JN, Chung KY, Cliby WA, Dorigo O, Eisen DB, Fujisawa Y, Golda N, Halfdanarson TR, Iavazzo C, Jiang SIB, Kanitakis J, Khan A, Kim JYS, Kuzel TM, Lawrence N, Leitao MM Jr, MacLean AB, Maher IA, Mittal BB, Nehal KS, Ozog DM, Pettaway CA, Ross JS, Rossi AM, Servaes S, Solomon MJ, Thomas VD, Tolia M, Voelzke BB, Waldman A, Wong MK, Zhou Y, Arai N, Brackett A, Ibrahim SA, Kang BY, Poon E, Alam M
JAMA Oncol 2022 Apr 1;8(4):618-628. doi: 10.1001/jamaoncol.2021.7148. PMID: 35050310
Longitudinal multicentre retrospective cohort study of treatment outcomes in extramammary Paget disease.
Liu KX, Huang V, Chen CA, Elco CP, Chen ST, Stern RS, Wu PA
Br J Dermatol 2021 Jul;185(1):219-221. Epub 2021 Jun 3 doi: 10.1111/bjd.19871. PMID: 33548065

Recent clinical studies

Etiology

Mutational landscape of advanced extramammary Paget disease with comprehensive genomic profiling tests: A cohort study in a Japanese real-world setting.
Iwasawa O, Miyagawa T, Omatsu J, Matsuda KM, Awaji K, Yamada D, Kage H, Oda K, Sato S
J Am Acad Dermatol 2024 Apr;90(4):842-845. Epub 2023 Dec 9 doi: 10.1016/j.jaad.2023.11.057. PMID: 38072126
Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.
Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, Aasi SZ, Aung PP, Barker CA, Bolotin D, Bordeaux JS, Cartee TV, Chandra S, Cho NL, Choi JN, Chung KY, Cliby WA, Dorigo O, Eisen DB, Fujisawa Y, Golda N, Halfdanarson TR, Iavazzo C, Jiang SIB, Kanitakis J, Khan A, Kim JYS, Kuzel TM, Lawrence N, Leitao MM Jr, MacLean AB, Maher IA, Mittal BB, Nehal KS, Ozog DM, Pettaway CA, Ross JS, Rossi AM, Servaes S, Solomon MJ, Thomas VD, Tolia M, Voelzke BB, Waldman A, Wong MK, Zhou Y, Arai N, Brackett A, Ibrahim SA, Kang BY, Poon E, Alam M
JAMA Oncol 2022 Apr 1;8(4):618-628. doi: 10.1001/jamaoncol.2021.7148. PMID: 35050310
Extramammary Paget Disease.
Marcoval J, Penín RM, Vidal A, Bermejo J
Actas Dermosifiliogr (Engl Ed) 2020 May;111(4):306-312. Epub 2020 Apr 9 doi: 10.1016/j.ad.2019.09.003. PMID: 32278442
Mohs Micrographic Surgery: Past, Present, and Future.
Cohen DK, Goldberg DJ
Dermatol Surg 2019 Mar;45(3):329-339. doi: 10.1097/DSS.0000000000001701. PMID: 30608296
Treatment of Malignant Cutaneous Adnexal Neoplasms.
Bernárdez C, Requena L
Actas Dermosifiliogr (Engl Ed) 2018 Jan-Feb;109(1):6-23. Epub 2017 Oct 6 doi: 10.1016/j.ad.2017.04.005. PMID: 28988763

Diagnosis

Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.
Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, Aasi SZ, Aung PP, Barker CA, Bolotin D, Bordeaux JS, Cartee TV, Chandra S, Cho NL, Choi JN, Chung KY, Cliby WA, Dorigo O, Eisen DB, Fujisawa Y, Golda N, Halfdanarson TR, Iavazzo C, Jiang SIB, Kanitakis J, Khan A, Kim JYS, Kuzel TM, Lawrence N, Leitao MM Jr, MacLean AB, Maher IA, Mittal BB, Nehal KS, Ozog DM, Pettaway CA, Ross JS, Rossi AM, Servaes S, Solomon MJ, Thomas VD, Tolia M, Voelzke BB, Waldman A, Wong MK, Zhou Y, Arai N, Brackett A, Ibrahim SA, Kang BY, Poon E, Alam M
JAMA Oncol 2022 Apr 1;8(4):618-628. doi: 10.1001/jamaoncol.2021.7148. PMID: 35050310
Extramammary Paget's Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments.
Ishizuki S, Nakamura Y
Curr Oncol 2021 Aug 5;28(4):2969-2986. doi: 10.3390/curroncol28040260. PMID: 34436026Free PMC Article
Penoscrotal Paget's disease.
Dauendorffer JN, Herms F, Baroudjian B, Basset-Seguin N, Cavelier-Balloy B, Fouéré S, Bagot M, Lebbé C
Ann Dermatol Venereol 2021 Jun;148(2):71-76. Epub 2021 Jan 16 doi: 10.1016/j.annder.2020.08.051. PMID: 33461789
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Extramammary Paget's disease.
Shepherd V, Davidson EJ, Davies-Humphreys J
BJOG 2005 Mar;112(3):273-9. doi: 10.1111/j.1471-0528.2004.00438.x. PMID: 15713139

Therapy

Patient's Experience With Imiquimod for Extramammary Paget Disease.
Leong JY, Shumaker A, D'mello D, Jones EK, Chung PH
Dermatol Surg 2023 Jul 1;49(7):706-707. Epub 2023 Apr 18 doi: 10.1097/DSS.0000000000003807. PMID: 37093691
Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.
Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, Aasi SZ, Aung PP, Barker CA, Bolotin D, Bordeaux JS, Cartee TV, Chandra S, Cho NL, Choi JN, Chung KY, Cliby WA, Dorigo O, Eisen DB, Fujisawa Y, Golda N, Halfdanarson TR, Iavazzo C, Jiang SIB, Kanitakis J, Khan A, Kim JYS, Kuzel TM, Lawrence N, Leitao MM Jr, MacLean AB, Maher IA, Mittal BB, Nehal KS, Ozog DM, Pettaway CA, Ross JS, Rossi AM, Servaes S, Solomon MJ, Thomas VD, Tolia M, Voelzke BB, Waldman A, Wong MK, Zhou Y, Arai N, Brackett A, Ibrahim SA, Kang BY, Poon E, Alam M
JAMA Oncol 2022 Apr 1;8(4):618-628. doi: 10.1001/jamaoncol.2021.7148. PMID: 35050310
Photodynamic therapy for extramammary Paget's disease: A systematic review of the literature.
Shim PJ, Zeitouni NC
Photodiagnosis Photodyn Ther 2020 Sep;31:101911. Epub 2020 Jul 6 doi: 10.1016/j.pdpdt.2020.101911. PMID: 32645437
Extramammary Paget Disease.
Merritt BG, Degesys CA, Brodland DG
Dermatol Clin 2019 Jul;37(3):261-267. Epub 2019 Apr 19 doi: 10.1016/j.det.2019.02.002. PMID: 31084720
Extramammary Paget's disease.
Shepherd V, Davidson EJ, Davies-Humphreys J
BJOG 2005 Mar;112(3):273-9. doi: 10.1111/j.1471-0528.2004.00438.x. PMID: 15713139

Prognosis

Extramammary Paget's Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments.
Ishizuki S, Nakamura Y
Curr Oncol 2021 Aug 5;28(4):2969-2986. doi: 10.3390/curroncol28040260. PMID: 34436026Free PMC Article
Extramammary Paget disease of the vulva.
Konstantinova AM, Kazakov DV
Semin Diagn Pathol 2021 Jan;38(1):50687. Epub 2020 Sep 8 doi: 10.1053/j.semdp.2020.09.007. PMID: 32921571
Extramammary Paget Disease.
Merritt BG, Degesys CA, Brodland DG
Dermatol Clin 2019 Jul;37(3):261-267. Epub 2019 Apr 19 doi: 10.1016/j.det.2019.02.002. PMID: 31084720
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Extramammary Paget's disease.
Shepherd V, Davidson EJ, Davies-Humphreys J
BJOG 2005 Mar;112(3):273-9. doi: 10.1111/j.1471-0528.2004.00438.x. PMID: 15713139

Clinical prediction guides

Perianal Paget's-an aggressive disease.
Rudnicki Y, Stapleton SM, Batra R, Gan T, Mathis KL, Kelley SR
Colorectal Dis 2023 Jun;25(6):1213-1221. Epub 2023 Mar 21 doi: 10.1111/codi.16549. PMID: 36945125
Extramammary Paget Disease.
Marcoval J, Penín RM, Vidal A, Bermejo J
Actas Dermosifiliogr (Engl Ed) 2020 May;111(4):306-312. Epub 2020 Apr 9 doi: 10.1016/j.ad.2019.09.003. PMID: 32278442
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Prognostic Factors of Extramammary Paget's Disease.
Hatta N
Curr Treat Options Oncol 2018 Aug 15;19(10):47. doi: 10.1007/s11864-018-0567-4. PMID: 30112691
Imiquimod 5% cream as a therapeutic option for extramammary Paget's disease.
Sawada M, Kato J, Yamashita T, Yoneta A, Hida T, Horimoto K, Sato S, Uhara H
J Dermatol 2018 Feb;45(2):216-219. Epub 2017 Nov 8 doi: 10.1111/1346-8138.14117. PMID: 29115681

Recent systematic reviews

Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.
Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, Aasi SZ, Aung PP, Barker CA, Bolotin D, Bordeaux JS, Cartee TV, Chandra S, Cho NL, Choi JN, Chung KY, Cliby WA, Dorigo O, Eisen DB, Fujisawa Y, Golda N, Halfdanarson TR, Iavazzo C, Jiang SIB, Kanitakis J, Khan A, Kim JYS, Kuzel TM, Lawrence N, Leitao MM Jr, MacLean AB, Maher IA, Mittal BB, Nehal KS, Ozog DM, Pettaway CA, Ross JS, Rossi AM, Servaes S, Solomon MJ, Thomas VD, Tolia M, Voelzke BB, Waldman A, Wong MK, Zhou Y, Arai N, Brackett A, Ibrahim SA, Kang BY, Poon E, Alam M
JAMA Oncol 2022 Apr 1;8(4):618-628. doi: 10.1001/jamaoncol.2021.7148. PMID: 35050310
Paget's disease of the vulva: A challenge for the gynaecologist.
Carton I, Lebreton M, Tesson C, Henno S, Lavoué V, Levêque J, Nyangoh-Timoh K
J Gynecol Obstet Hum Reprod 2021 Jan;50(1):101896. Epub 2020 Aug 20 doi: 10.1016/j.jogoh.2020.101896. PMID: 32828871
Photodynamic therapy for extramammary Paget's disease: A systematic review of the literature.
Shim PJ, Zeitouni NC
Photodiagnosis Photodyn Ther 2020 Sep;31:101911. Epub 2020 Jul 6 doi: 10.1016/j.pdpdt.2020.101911. PMID: 32645437
Nonsurgical Treatments for Extramammary Paget Disease: A Systematic Review and Meta-Analysis.
Snast I, Sharon E, Kaftory R, Noyman Y, Oren-Shabtai M, Lapidoth M, Hodak E, Mimouni D, Mazor S, Levi A
Dermatology 2020;236(6):493-499. Epub 2020 Apr 14 doi: 10.1159/000506832. PMID: 32289785
Effects of imiquimod on vulvar Paget's disease: a systematic review of literature.
Machida H, Moeini A, Roman LD, Matsuo K
Gynecol Oncol 2015 Oct;139(1):165-71. Epub 2015 Jul 17 doi: 10.1016/j.ygyno.2015.07.097. PMID: 26193428Free PMC Article

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