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Scrotum Paget disease

MedGen UID:
65959
•Concept ID:
C0238330
•
Disease or Syndrome
Synonyms: Paget Disease of the Scrotum; Paget disease of the scrotum; Paget's Disease of Scrotum; Paget's disease of scrotum; Paget's Disease of the Scrotum; Paget's disease of the scrotum; Scrotal Paget Disease; Scrotal Paget's Disease; scrotal Paget's disease; scrotum mammary Paget's disease; scrotum Paget disease; scrotum Paget's disease
 
Monarch Initiative: MONDO:0002649

Definition

A rare form of Paget disease that arises from the scrotum. It is usually not associated with an underlying malignancy. It presents as a red plaque or raised lesion. Microscopically, it is characterized by the presence of the typical Paget cells which are large, round cells with abundant cytoplasm and prominent nuclei. [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
Surgical Management of Primary Scrotal Cancer.
Huang JH, Broggi M, Osunkoya AO, Master VA
Urol Clin North Am 2016 Nov;43(4):531-544. Epub 2016 Sep 3 doi: 10.1016/j.ucl.2016.06.014. PMID: 27717439
Preneoplastic and Primary Scrotal Cancer: Updates on Pathogenesis and Diagnostic Evaluation.
Zhu Y, Ye DW
Urol Clin North Am 2016 Nov;43(4):523-530. doi: 10.1016/j.ucl.2016.06.013. PMID: 27717438

Recent clinical studies

Etiology

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
P16 Expression in Extramammary Paget's Disease of the Vulva and Scrotum Is Not Human Papillomavirus Related.
Al-Obaidy KI, Kao CS, Idrees MT
Int J Surg Pathol 2018 Oct;26(7):617-620. Epub 2018 May 10 doi: 10.1177/1066896918775513. PMID: 29745285
Surgical Management of Primary Scrotal Cancer.
Huang JH, Broggi M, Osunkoya AO, Master VA
Urol Clin North Am 2016 Nov;43(4):531-544. Epub 2016 Sep 3 doi: 10.1016/j.ucl.2016.06.014. PMID: 27717439
Extramammary Paget's disease of scrotum--report of 25 cases and literature review.
Zhang N, Gong K, Zhang X, Yang Y, Na Y
Urol Oncol 2010 Jan-Feb;28(1):28-33. Epub 2008 Sep 21 doi: 10.1016/j.urolonc.2008.07.002. PMID: 18805708

Diagnosis

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
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
Extramammary Paget disease.
Hartman R, Chu J, Patel R, Meehan S, Stein JA
Dermatol Online J 2011 Oct 15;17(10):4. PMID: 22031630

Therapy

Comparison of the biomarkers for targeted therapies in primary extra-mammary and mammary Paget's disease.
Gatalica Z, Vranic S, Krušlin B, Poorman K, Stafford P, Kacerovska D, Senarathne W, Florento E, Contreras E, Leary A, Choi A, In GK
Cancer Med 2020 Feb;9(4):1441-1450. Epub 2020 Jan 3 doi: 10.1002/cam4.2820. PMID: 31899853Free PMC Article
Excision combined with photodynamic therapy for scrotal Paget's disease in patients aged over 60 years.
Chen M, Chen X, Dai Y, Yang Z, Zhang X, Li D
Aging Male 2020 Dec;23(5):854-859. Epub 2019 Apr 29 doi: 10.1080/13685538.2019.1607284. PMID: 31033366
Combination Cisplatin-Epirubicin-Paclitaxel Therapy for Metastatic Extramammary Paget's Disease.
Hirai I, Tanese K, Nakamura Y, Ishii M, Kawakami Y, Funakoshi T
Oncologist 2019 Jun;24(6):e394-e396. Epub 2019 Mar 7 doi: 10.1634/theoncologist.2018-0856. PMID: 30846514Free PMC Article
Extramammary Paget disease.
Hartman R, Chu J, Patel R, Meehan S, Stein JA
Dermatol Online J 2011 Oct 15;17(10):4. PMID: 22031630
Extramammary Paget's disease.
Haberman HF, Goodall J, Llewellyn M
Can Med Assoc J 1978 Jan 21;118(2):161-2. PMID: 230891Free PMC Article

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.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Surgical Management of Primary Scrotal Cancer.
Huang JH, Broggi M, Osunkoya AO, Master VA
Urol Clin North Am 2016 Nov;43(4):531-544. Epub 2016 Sep 3 doi: 10.1016/j.ucl.2016.06.014. PMID: 27717439
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
Extramammary Paget's disease of scrotum--report of 25 cases and literature review.
Zhang N, Gong K, Zhang X, Yang Y, Na Y
Urol Oncol 2010 Jan-Feb;28(1):28-33. Epub 2008 Sep 21 doi: 10.1016/j.urolonc.2008.07.002. PMID: 18805708

Clinical prediction guides

TRPS1 expression is sensitive and specific for primary extramammary Paget disease.
Cook EE, Harrison BT, Hirsch MS
Histopathology 2023 Jul;83(1):104-108. Epub 2023 Mar 27 doi: 10.1111/his.14908. PMID: 36971374
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
A clinical series and literature review of the management of inguinal nodal metastases in patients with primary extramammary Paget disease of the scrotum.
Koh YX, Tay TK, Xu S, Lee CM, Teo MC
Asian J Surg 2015 Jan;38(1):40-6. Epub 2014 Oct 16 doi: 10.1016/j.asjsur.2014.08.001. PMID: 25444176

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