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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
Vulvar Paget's disease: outcomes of 51 patients treated with imiquimod cream.
Borella F, Preti M, Vieira-Baptista P, Pérez-López FR, Bertero L, Gallio N, Micheletti L, Benedetto C
Maturitas 2022 Sep;163:23-27. Epub 2022 May 27 doi: 10.1016/j.maturitas.2022.05.010. PMID: 35661559
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 disease of the vulva.
Johnson J, OʼLaughlin D
JAAPA 2019 Mar;32(3):33-34. doi: 10.1097/01.JAA.0000550285.65463.a9. PMID: 30817477
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

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
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
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
Vulvar Cancer.
Weinberg D, Gomez-Martinez RA
Obstet Gynecol Clin North Am 2019 Mar;46(1):125-135. doi: 10.1016/j.ogc.2018.09.008. PMID: 30683259
Paget disease of the vulva.
van der Linden M, Meeuwis KA, Bulten J, Bosse T, van Poelgeest MI, de Hullu JA
Crit Rev Oncol Hematol 2016 May;101:60-74. Epub 2016 Mar 4 doi: 10.1016/j.critrevonc.2016.03.008. PMID: 26971063

Therapy

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
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
Uncommon anal neoplasms.
Billingsley KG, Stern LE, Lowy AM, Kahlenberg MS, Thomas CR Jr
Surg Oncol Clin N Am 2004 Apr;13(2):375-88. doi: 10.1016/j.soc.2003.12.002. PMID: 15137963

Prognosis

Pedunculated Neoplasm of the Vulva.
Zeng Z, Xiong X, Liu Z, Lei Q, He Y
Am J Dermatopathol 2024 Oct 1;46(10):693-695. Epub 2024 Jun 14 doi: 10.1097/DAD.0000000000002764. PMID: 38916239
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
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
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Acquired disorders with depigmentation: A systematic approach to vitiliginoid conditions.
Saleem MD, Oussedik E, Schoch JJ, Berger AC, Picardo M
J Am Acad Dermatol 2019 May;80(5):1215-1231.e6. Epub 2018 Sep 17 doi: 10.1016/j.jaad.2018.03.063. PMID: 30236516

Clinical prediction guides

Vulvar Paget's disease: outcomes of 51 patients treated with imiquimod cream.
Borella F, Preti M, Vieira-Baptista P, Pérez-López FR, Bertero L, Gallio N, Micheletti L, Benedetto C
Maturitas 2022 Sep;163:23-27. Epub 2022 May 27 doi: 10.1016/j.maturitas.2022.05.010. PMID: 35661559
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
Pagetoid dyskeratosis in dermatopathology.
Santos-Briz A, Cañueto J, del Carmen S, Mir-Bonafe JM, Fernandez E
Am J Dermatopathol 2015 Apr;37(4):261-5; quiz 266-8. doi: 10.1097/DAD.0000000000000284. PMID: 25794368
Skin cancer incidence among atomic bomb survivors from 1958 to 1996.
Sugiyama H, Misumi M, Kishikawa M, Iseki M, Yonehara S, Hayashi T, Soda M, Tokuoka S, Shimizu Y, Sakata R, Grant EJ, Kasagi F, Mabuchi K, Suyama A, Ozasa K
Radiat Res 2014 May;181(5):531-9. Epub 2014 Apr 22 doi: 10.1667/RR13494.1. PMID: 24754560

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
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

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