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Paget disease of the penis

MedGen UID:
66374
•Concept ID:
C0221286
•
Neoplastic Process
Synonyms: adenocarcinoma of penis; adenocarcinoma of the penis; Paget Disease of the Penis; Paget's Disease of Penis; Paget's disease of penis; Paget's Disease of the Penis; Paget's disease of the penis; penile adenocarcinoma; Penile Paget Disease; penis adenocarcinoma; penis mammary Paget's disease; penis Paget disease; penis Paget's disease
SNOMED CT: Paget's disease of penis (398768004)
 
Monarch Initiative: MONDO:0002653

Definition

A rare, slow-growing, usually non-invasive intraepithelial adenocarcinoma affecting the penile skin or mucosal surface. The malignant cells are large with abundant pale cytoplasm and vesicular nuclei with prominent nucleoli. (WHO 2016) [from NCI]

Professional guidelines

PubMed

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
Multidisciplinary Approach for the Management of Penoscrotal Extramammary Paget's disease -An eUROGEN study.
Christodoulidou M, Alnajjar HM, Parnham A, Khetrapal P, Freeman A, Haider A, Mitra AV, Bunker CB, Muneer A
Urol Oncol 2021 Aug;39(8):501.e1-501.e10. Epub 2021 Jun 27 doi: 10.1016/j.urolonc.2021.05.018. PMID: 34193377
Cancers, precancers, and pseudocancers on the male genitalia. A review of clinical appearances, histopathology, and management.
Mikhail GR
J Dermatol Surg Oncol 1980 Dec;6(12):1027-35. doi: 10.1111/j.1524-4725.1980.tb01026.x. PMID: 6259230

Recent clinical studies

Etiology

Multidisciplinary Approach for the Management of Penoscrotal Extramammary Paget's disease -An eUROGEN study.
Christodoulidou M, Alnajjar HM, Parnham A, Khetrapal P, Freeman A, Haider A, Mitra AV, Bunker CB, Muneer A
Urol Oncol 2021 Aug;39(8):501.e1-501.e10. Epub 2021 Jun 27 doi: 10.1016/j.urolonc.2021.05.018. PMID: 34193377
A population-level analysis of nonsquamous penile cancer: The importance of histology.
Bhambhvani HP, Greenberg DR, Parham MJ, Eisenberg ML
Urol Oncol 2021 Feb;39(2):136.e1-136.e10. Epub 2020 Nov 27 doi: 10.1016/j.urolonc.2020.11.025. PMID: 33257222
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
Efficacy and safety of topical ALA-PDT in the treatment of EMPD.
Gao Y, Zhang XC, Wang WS, Yang Y, Wang HL, Lu YG, Fan DL
Photodiagnosis Photodyn Ther 2015 Mar;12(1):92-7. Epub 2014 Dec 3 doi: 10.1016/j.pdpdt.2014.11.004. PMID: 25479094
Malignant tumors of the penis.
Brady KL, Mercurio MG, Brown MD
Dermatol Surg 2013 Apr;39(4):527-47. Epub 2012 Nov 16 doi: 10.1111/dsu.12029. PMID: 23164051

Diagnosis

Extra-mammary Paget's disease of the penis.
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
Extramammary paget disease of the penoscrotal region.
Patwardhan PP, Quiroga-Garza GM
Pathol Res Pract 2023 Jan;241:154283. Epub 2022 Dec 22 doi: 10.1016/j.prp.2022.154283. PMID: 36584497
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

Therapy

Efficacy and safety of HpD-PDT for Extramammary Paget's Disease refractory to conventional therapy: A prospective, open-label and single arm pilot study.
Wang D, Wang P, Li C, Zhou Z, Zhang L, Zhang G, Wang X
Photodiagnosis Photodyn Ther 2022 Mar;37:102670. Epub 2021 Dec 7 doi: 10.1016/j.pdpdt.2021.102670. PMID: 34883272
Boron neutron capture therapy for vulvar melanoma and genital extramammary Paget's disease with curative responses.
Hiratsuka J, Kamitani N, Tanaka R, Yoden E, Tokiya R, Suzuki M, Barth RF, Ono K
Cancer Commun (Lond) 2018 Jun 19;38(1):38. doi: 10.1186/s40880-018-0297-9. PMID: 29914570Free PMC Article
Aesthetic Penoscrotal Resurfacing: Creating Propeller Flaps from Gluteal Folds.
Han SE, Kim EJ, Sung HH, Pyon JK
Eur Urol 2018 Apr;73(4):610-617. Epub 2016 Oct 12 doi: 10.1016/j.eururo.2016.09.033. PMID: 27743757
Efficacy and safety of topical ALA-PDT in the treatment of EMPD.
Gao Y, Zhang XC, Wang WS, Yang Y, Wang HL, Lu YG, Fan DL
Photodiagnosis Photodyn Ther 2015 Mar;12(1):92-7. Epub 2014 Dec 3 doi: 10.1016/j.pdpdt.2014.11.004. PMID: 25479094
Successful treatment of extramammary Paget's disease with imiquimod.
Qian Z, Zeitoun NC, Shieh S, Helm T, Oseroff AR
J Drugs Dermatol 2003 Jan;2(1):73-6. PMID: 12852386

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
Multidisciplinary Approach for the Management of Penoscrotal Extramammary Paget's disease -An eUROGEN study.
Christodoulidou M, Alnajjar HM, Parnham A, Khetrapal P, Freeman A, Haider A, Mitra AV, Bunker CB, Muneer A
Urol Oncol 2021 Aug;39(8):501.e1-501.e10. Epub 2021 Jun 27 doi: 10.1016/j.urolonc.2021.05.018. PMID: 34193377
A population-level analysis of nonsquamous penile cancer: The importance of histology.
Bhambhvani HP, Greenberg DR, Parham MJ, Eisenberg ML
Urol Oncol 2021 Feb;39(2):136.e1-136.e10. Epub 2020 Nov 27 doi: 10.1016/j.urolonc.2020.11.025. PMID: 33257222
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Mammary and extramammary Paget's disease.
Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N
An Bras Dermatol 2015 Mar-Apr;90(2):225-31. doi: 10.1590/abd1806-4841.20153189. PMID: 25830993Free PMC Article

Clinical prediction guides

Electron radiotherapy for EMPD of penis and scrotum.
Lee HK, Youn SM, Shim SJ, Lee MJ
J Cancer Res Ther 2023 Jan 1;19(Suppl 2):S998-S1001. Epub 2023 Apr 28 doi: 10.4103/jcrt.jcrt_506_21. PMID: 38384101
A population-level analysis of nonsquamous penile cancer: The importance of histology.
Bhambhvani HP, Greenberg DR, Parham MJ, Eisenberg ML
Urol Oncol 2021 Feb;39(2):136.e1-136.e10. Epub 2020 Nov 27 doi: 10.1016/j.urolonc.2020.11.025. PMID: 33257222
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's disease of scrotum and penis case report of a re-irradiation and review of the literature.
Piras A, Sanfratello A, Boldrini L, La Vecchia M, Venuti V, Amari ML, Orlando M, Zichichi L, Angileri T, Daidone A
Dermatol Ther 2020 Nov;33(6):e13890. Epub 2020 Jul 25 doi: 10.1111/dth.13890. PMID: 32584449
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904

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