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Paget disease

MedGen UID:
277958
•Concept ID:
C1368019
•
Neoplastic Process
Synonyms: Paget Cell Neoplasm; Paget Disease; Paget's Cell Neoplasm; Paget's cell neoplasm; Paget's Disease; Paget's disease
 
Monarch Initiative: MONDO:0021165

Definition

A malignant neoplasm composed of large cells with large nuclei, prominent nucleoli, and abundant pale cytoplasm (Paget cells). Paget cell neoplasms include Paget disease of the nipple and extramammary Paget disease which may affect the vulva, penis, anus, skin and scrotum. [from NCI]

Professional guidelines

PubMed

Breast Cancer, Version 3.2022, NCCN Clinical Practice Guidelines in Oncology.
Gradishar WJ, Moran MS, Abraham J, Aft R, Agnese D, Allison KH, Anderson B, Burstein HJ, Chew H, Dang C, Elias AD, Giordano SH, Goetz MP, Goldstein LJ, Hurvitz SA, Isakoff SJ, Jankowitz RC, Javid SH, Krishnamurthy J, Leitch M, Lyons J, Mortimer J, Patel SA, Pierce LJ, Rosenberger LH, Rugo HS, Sitapati A, Smith KL, Smith ML, Soliman H, Stringer-Reasor EM, Telli ML, Ward JH, Wisinski KB, Young JS, Burns J, Kumar R
J Natl Compr Canc Netw 2022 Jun;20(6):691-722. doi: 10.6004/jnccn.2022.0030. PMID: 35714673
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
Diagnosis and management of vulvar cancer: A review.
Tan A, Bieber AK, Stein JA, Pomeranz MK
J Am Acad Dermatol 2019 Dec;81(6):1387-1396. Epub 2019 Jul 23 doi: 10.1016/j.jaad.2019.07.055. PMID: 31349045

Recent clinical studies

Etiology

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.
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
Spinal stenosis.
Melancia JL, Francisco AF, Antunes JL
Handb Clin Neurol 2014;119:541-9. doi: 10.1016/B978-0-7020-4086-3.00035-7. PMID: 24365318
Osteosarcoma incidence and survival rates from 1973 to 2004: data from the Surveillance, Epidemiology, and End Results Program.
Mirabello L, Troisi RJ, Savage SA
Cancer 2009 Apr 1;115(7):1531-43. doi: 10.1002/cncr.24121. PMID: 19197972Free PMC Article

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
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
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
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
Bisphosphonates: mechanism of action and role in clinical practice.
Drake MT, Clarke BL, Khosla S
Mayo Clin Proc 2008 Sep;83(9):1032-45. doi: 10.4065/83.9.1032. PMID: 18775204Free 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.
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
Treatment of Mammary Paget Disease: A systematic review and meta-analysis of real-world data.
Lin CW, Chiang MH, Tam KW
Int J Surg 2022 Nov;107:106964. Epub 2022 Oct 26 doi: 10.1016/j.ijsu.2022.106964. PMID: 36309195
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
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS.
Kim HJ, Kim NC, Wang YD, Scarborough EA, Moore J, Diaz Z, MacLea KS, Freibaum B, Li S, Molliex A, Kanagaraj AP, Carter R, Boylan KB, Wojtas AM, Rademakers R, Pinkus JL, Greenberg SA, Trojanowski JQ, Traynor BJ, Smith BN, Topp S, Gkazi AS, Miller J, Shaw CE, Kottlors M, Kirschner J, Pestronk A, Li YR, Ford AF, Gitler AD, Benatar M, King OD, Kimonis VE, Ross ED, Weihl CC, Shorter J, Taylor JP
Nature 2013 Mar 28;495(7442):467-73. Epub 2013 Mar 3 doi: 10.1038/nature11922. PMID: 23455423Free PMC Article

Recent systematic reviews

Treatment of Mammary Paget Disease: A systematic review and meta-analysis of real-world data.
Lin CW, Chiang MH, Tam KW
Int J Surg 2022 Nov;107:106964. Epub 2022 Oct 26 doi: 10.1016/j.ijsu.2022.106964. PMID: 36309195
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
Mammary Paget's disease and radiotherapy: a systematic literature review.
Piras A, Boldrini L, Venuti V, Sanfratello A, La Vecchia M, Gennari R, Sortino G, Angileri T, Daidone A
Eur Rev Med Pharmacol Sci 2021 Feb;25(4):1821-1827. doi: 10.26355/eurrev_202102_25076. PMID: 33660791
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
Effectiveness of photodynamic therapy for mammary and extra-mammary Paget's disease: a state of the science review.
Nardelli AA, Stafinski T, Menon D
BMC Dermatol 2011 Jun 15;11:13. doi: 10.1186/1471-5945-11-13. PMID: 21676258Free PMC Article

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