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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
Longitudinal multicentre retrospective cohort study of treatment outcomes in extramammary Paget disease.
Liu KX, Huang V, Chen CA, Elco CP, Chen ST, Stern RS, Wu PA
Br J Dermatol 2021 Jul;185(1):219-221. Epub 2021 Jun 3 doi: 10.1111/bjd.19871. PMID: 33548065

Recent clinical studies

Etiology

Metabolic Bone Disease.
Seemann LL, Hanos CT, Pujalte GGA
Prim Care 2024 Sep;51(3):445-454. Epub 2024 May 23 doi: 10.1016/j.pop.2024.04.005. PMID: 39067970
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
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
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
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
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
Perianal Paget disease.
Merichal Resina M, Cerdan Santacruz C, Sierra Grañón E, Tarragona Foradada JA, Olsina Kissler JJ
Cir Esp (Engl Ed) 2019 Mar;97(3):179-180. Epub 2018 Sep 18 doi: 10.1016/j.ciresp.2018.08.001. PMID: 30241672
Extramammary Paget's disease.
Shepherd V, Davidson EJ, Davies-Humphreys J
BJOG 2005 Mar;112(3):273-9. doi: 10.1111/j.1471-0528.2004.00438.x. PMID: 15713139

Therapy

Metabolic Bone Disease.
Seemann LL, Hanos CT, Pujalte GGA
Prim Care 2024 Sep;51(3):445-454. Epub 2024 May 23 doi: 10.1016/j.pop.2024.04.005. PMID: 39067970
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
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
Extramammary Paget's disease.
Shepherd V, Davidson EJ, Davies-Humphreys J
BJOG 2005 Mar;112(3):273-9. doi: 10.1111/j.1471-0528.2004.00438.x. PMID: 15713139

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 of the vulva.
Konstantinova AM, Kazakov DV
Semin Diagn Pathol 2021 Jan;38(1):50687. Epub 2020 Sep 8 doi: 10.1053/j.semdp.2020.09.007. PMID: 32921571
Extramammary Paget disease.
St Claire K, Hoover A, Ashack K, Khachemoune A
Dermatol Online J 2019 Apr 15;25(4) PMID: 31046904
Imaging of Paget disease of bone and its musculoskeletal complications: review.
Theodorou DJ, Theodorou SJ, Kakitsubata Y
AJR Am J Roentgenol 2011 Jun;196(6 Suppl):S64-75. doi: 10.2214/AJR.10.7222. PMID: 21606236
Extramammary Paget's disease.
Shepherd V, Davidson EJ, Davies-Humphreys J
BJOG 2005 Mar;112(3):273-9. doi: 10.1111/j.1471-0528.2004.00438.x. PMID: 15713139

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
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
Advanced Paget disease of bone.
Choy KH, Alukaidey S
Med J Aust 2017 Aug 21;207(4):146. doi: 10.5694/mja17.00303. PMID: 28814210
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
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

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