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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]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVScrotum Paget disease

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

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
External beam radiotherapy of extramammary Paget disease.
Tackenberg S, Gehrig A, Dummer R, Navarini AA
Cutis 2015 Feb;95(2):109-12. PMID: 25750964
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
Genital Paget's disease in a man.
Krause W, Krisp A, Hörster S, Hoffmann R
Eur J Dermatol 2006 Jan-Feb;16(1):75-8. PMID: 16436348
Extramammary Paget's disease.
Mehta NJ, Torno R, Sorra T
South Med J 2000 Jul;93(7):713-5. PMID: 10923963

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
Bilateral Contiguous Scrotal Extramammary Paget's Disease Treated With Mohs Micrographic Surgery and CK7 Immunohistochemical Staining.
Kim EY, Nadimi AE, Bruno JR, Hendi A
J Drugs Dermatol 2021 May 1;20(5):565-566. doi: 10.36849/JDD.5512. PMID: 33938703
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
Erythematous Velvety Plaque of the Scrotum and Penis.
Law YXT, Gan VHL, Tay KJ
JAMA Oncol 2018 Jun 1;4(6):861-862. doi: 10.1001/jamaoncol.2017.4064. PMID: 29270606

Therapy

Advanced Scrotal Extramammary Paget's Disease Treated With Apatinib: A Case Report.
Zhang YN, Chen Y, Gao F, Chen N, Liu JY
Clin Genitourin Cancer 2018 Apr;16(2):e339-e342. Epub 2017 Oct 16 doi: 10.1016/j.clgc.2017.10.002. PMID: 29113769
Extramammary Paget disease.
Hartman R, Chu J, Patel R, Meehan S, Stein JA
Dermatol Online J 2011 Oct 15;17(10):4. PMID: 22031630
Genital Paget's disease in a man.
Krause W, Krisp A, Hörster S, Hoffmann R
Eur J Dermatol 2006 Jan-Feb;16(1):75-8. PMID: 16436348
Radiation-associated venous stenosis: endovascular treatment options.
Zhou W, Bush RL, Lin PH, Lumsden AB
J Vasc Surg 2004 Jul;40(1):179-82. doi: 10.1016/j.jvs.2004.03.039. PMID: 15218482
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.
Mehta NJ, Torno R, Sorra T
South Med J 2000 Jul;93(7):713-5. PMID: 10923963

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
Imaging Features of Extramammary Paget Disease of the Scrotum: A Case Report.
Choi SL, Park SB, Lee ES, Park HJ, Lee JB, Choi BI
Ultrasound Q 2020 Mar;36(1):84-86. doi: 10.1097/RUQ.0000000000000362. PMID: 29727346
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
Successful treatment of extramammary Paget's disease of the scrotum with imiquimod 5% cream.
Berman B, Spencer J, Villa A, Poochareon V, Elgart G
Clin Exp Dermatol 2003 Nov;28 Suppl 1:36-8. doi: 10.1046/j.1365-2230.28.s1.12.x. PMID: 14616812

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