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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

Extramammary Paget disease. Part II. Evidence-based approach to management.
Shah RR, Shah K, Wilson BN, Leitao MM, Smogorzewski J, Nguyen KA, Crane C, Funt SA, Hosein S, Dafinone M, Rossi A
J Am Acad Dermatol 2024 Sep;91(3):421-430. Epub 2024 Apr 6 doi: 10.1016/j.jaad.2023.07.1052. PMID: 38588817
EXTRA, EXTRA, Treatment Approaches for EXTRAmammary Paget Disease.
Desai S, McCormick E, Nelson K, Friedman A
J Drugs Dermatol 2023 Aug 1;22(8):844-846. doi: 10.36849/jdd.0823. PMID: 37556527
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

Recent clinical studies

Etiology

Vulvar Cancer, Version 3.2024, NCCN Clinical Practice Guidelines in Oncology.
Abu-Rustum NR, Yashar CM, Arend R, Barber E, Bradley K, Brooks R, Campos SM, Chino J, Chon HS, Crispens MA, Damast S, Fisher CM, Frederick P, Gaffney DK, Gaillard S, Giuntoli R II, Glaser S, Holmes J, Howitt BE, Kendra K, Lea J, Lee N, Mantia-Smaldone G, Mariani A, Mutch D, Nagel C, Nekhlyudov L, Podoll M, Rodabaugh K, Salani R, Schorge J, Siedel J, Sisodia R, Soliman P, Ueda S, Urban R, Wethington SL, Wyse E, Zanotti K, McMillian N, Espinosa S
J Natl Compr Canc Netw 2024 Mar;22(2):117-135. doi: 10.6004/jnccn.2024.0013. PMID: 38503056
The European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of Vulval Disease (ECSVD) and the European Federation for Colposcopy (EFC) Consensus Statements on Pre-invasive Vulvar Lesions.
Preti M, Joura E, Vieira-Baptista P, Van Beurden M, Bevilacqua F, Bleeker MCG, Bornstein J, Carcopino X, Chargari C, Cruickshank ME, Erzeneoglu BE, Gallio N, Heller D, Kesic V, Reich O, Stockdale CK, Temiz BE, Woelber L, Planchamp F, Zodzika J, Querleu D, Gultekin M
J Low Genit Tract Dis 2022 Jul 1;26(3):229-244. Epub 2022 Jun 21 doi: 10.1097/LGT.0000000000000683. PMID: 35763611Free PMC Article
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 of genitourinary origin.
Narayan VM, Master VA
Curr Opin Urol 2022 Mar 1;32(2):211-215. doi: 10.1097/MOU.0000000000000948. PMID: 34798637
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

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
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.
Asel M, LeBoeuf NR
Hematol Oncol Clin North Am 2019 Feb;33(1):73-85. doi: 10.1016/j.hoc.2018.09.003. PMID: 30497678

Therapy

The European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of Vulval Disease (ECSVD) and the European Federation for Colposcopy (EFC) Consensus Statements on Pre-invasive Vulvar Lesions.
Preti M, Joura E, Vieira-Baptista P, Van Beurden M, Bevilacqua F, Bleeker MCG, Bornstein J, Carcopino X, Chargari C, Cruickshank ME, Erzeneoglu BE, Gallio N, Heller D, Kesic V, Reich O, Stockdale CK, Temiz BE, Woelber L, Planchamp F, Zodzika J, Querleu D, Gultekin M
J Low Genit Tract Dis 2022 Jul 1;26(3):229-244. Epub 2022 Jun 21 doi: 10.1097/LGT.0000000000000683. PMID: 35763611Free PMC Article
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.
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
The diagnosis and management of extramammary Paget's disease.
Ito T, Kaku-Ito Y, Furue M
Expert Rev Anticancer Ther 2018 Jun;18(6):543-553. Epub 2018 Mar 27 doi: 10.1080/14737140.2018.1457955. PMID: 29575944
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.
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.
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.
Asel M, LeBoeuf NR
Hematol Oncol Clin North Am 2019 Feb;33(1):73-85. doi: 10.1016/j.hoc.2018.09.003. PMID: 30497678

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's disease: what do we know and how do we treat?
Adashek JJ, Leonard A, Nealon SW, Krishnan A, Mosiello GC, Dhillon J, Spiess PE
Can J Urol 2019 Dec;26(6):10012-10021. PMID: 31860417
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

Recent systematic reviews

The role of sentinel lymph node biopsy in extramammary paget disease: a systematic review.
Faisel L, Swanson A, Sheridan C, Walker T, Carr DR, Shahwan KT
Arch Dermatol Res 2023 Nov;315(9):2513-2518. Epub 2023 Jun 2 doi: 10.1007/s00403-023-02649-8. PMID: 37266674
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
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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