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Anal margin Paget disease

MedGen UID:
231372
•Concept ID:
C1332270
•
Neoplastic Process
Synonym: Anal margin Paget's disease
 
HPO: HP:0030441
Monarch Initiative: MONDO:0004337

Definition

An intraepithelial adenocarcinoma originating in the anal margin and characterized by presence of typical Paget's cells, appearing as large rounded vacuolated cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnal margin Paget disease

Professional guidelines

PubMed

Extra-mammary Paget's disease of the perianal region: a review of the literature emphasizing the operative management technique.
Kyriazanos ID, Stamos NP, Miliadis L, Noussis G, Stoidis CN
Surg Oncol 2011 Jun;20(2):e61-71. doi: 10.1016/j.suronc.2010.09.005. PMID: 20884199
Management of less common tumors of the colon, rectum, and anus.
Cuffy M, Abir F, Longo WE
Clin Colorectal Cancer 2006 Jan;5(5):327-37. doi: 10.3816/CCC.2006.n.002. PMID: 16512991
Conservative treatment by irradiation of epidermoid carcinomas of the anal margin.
Peiffert D, Bey P, Pernot M, Hoffstetter S, Marchal C, Beckendorf V, Guillemin F
Int J Radiat Oncol Biol Phys 1997 Aug 1;39(1):57-66. doi: 10.1016/s0360-3016(97)00316-7. PMID: 9300740

Recent clinical studies

Etiology

Clinical features of pagetoid spread: Analysis of perianal lesions associated with anal canal adenocarcinoma and perianal primary extramammary Paget disease.
Kosano M, Kiniwa Y, Mikoshiba A, Iwaya M, Okuyama R
J Dermatol 2023 Apr;50(4):546-550. Epub 2023 Feb 16 doi: 10.1111/1346-8138.16744. PMID: 36794662
Perianal Paget disease treated with wide excision and thigh skin flap reconstruction: a case report and review of literature.
Shen K, Luo H, Hu J, Xie Z
Medicine (Baltimore) 2018 Jul;97(30):e11638. doi: 10.1097/MD.0000000000011638. PMID: 30045309Free PMC Article
Perianal Paget's disease: three decades experience of a single institution.
Isik O, Aytac E, Brainard J, Valente MA, Abbas MA, Gorgun E
Int J Colorectal Dis 2016 Jan;31(1):29-34. Epub 2015 Aug 12 doi: 10.1007/s00384-015-2342-3. PMID: 26260479
Staged marginal contoured and central excision technique in the surgical management of perianal Paget's disease.
Möller MG, Lugo-Baruqui JA, Milikowski C, Salgado CJ
Am J Surg 2014 Apr;207(4):485-92. Epub 2013 Sep 20 doi: 10.1016/j.amjsurg.2013.04.005. PMID: 24055144
Paget's disease of the anal margin.
Jensen SL, Sjølin KE, Shokouh-Amiri MH, Hagen K, Harling H
Br J Surg 1988 Nov;75(11):1089-92. doi: 10.1002/bjs.1800751113. PMID: 2850073

Diagnosis

Perianal Paget's Disease.
Thompson HM, Kim JK
Dis Colon Rectum 2021 May;64(5):511-515. doi: 10.1097/DCR.0000000000002000. PMID: 33661234Free PMC Article
Perianal Paget disease treated with wide excision and thigh skin flap reconstruction: a case report and review of literature.
Shen K, Luo H, Hu J, Xie Z
Medicine (Baltimore) 2018 Jul;97(30):e11638. doi: 10.1097/MD.0000000000011638. PMID: 30045309Free PMC Article
Tumors and Tumorlike Conditions of the Anal Canal and Perianal Region: MR Imaging Findings.
Surabhi VR, Menias CO, Amer AM, Elshikh M, Katabathina VS, Hara AK, Baughman WC, Kielar A, Elsayes KM, Siegel CL
Radiographics 2016 Sep-Oct;36(5):1339-53. doi: 10.1148/rg.2016150209. PMID: 27618320
Perianal Paget's disease: case report and review of the literature.
Delaunoit T, Neczyporenko F, Duttmann R, Deprez C, da Costa PM, de Koster E
Acta Gastroenterol Belg 2004 Apr-Jun;67(2):228-31. PMID: 15285581
Paget's disease of the anal margin.
Jensen SL, Sjølin KE, Shokouh-Amiri MH, Hagen K, Harling H
Br J Surg 1988 Nov;75(11):1089-92. doi: 10.1002/bjs.1800751113. PMID: 2850073

Therapy

Perianal Paget's Disease.
Thompson HM, Kim JK
Dis Colon Rectum 2021 May;64(5):511-515. doi: 10.1097/DCR.0000000000002000. PMID: 33661234Free PMC Article
Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy.
Dos Santos JS, Bonafé GA, Pereira JA, Kanno DT, Martinez CAR, Ortega MM
BMC Cancer 2018 Sep 25;18(1):921. doi: 10.1186/s12885-018-4815-6. PMID: 30253739Free PMC Article
Perianal extramammary Paget disease treated with topical imiquimod and oral cimetidine.
Gray J, Gray A, Swan J, Eberhardt J, Mudaliar K, Tung R
Cutis 2018 Apr;101(4):E19-E22. PMID: 29763492
Photodynamic therapy in the management of neoplasms of the perianal skin.
Petrelli NJ, Cebollero JA, Rodriguez-Bigas M, Mang T
Arch Surg 1992 Dec;127(12):1436-8. doi: 10.1001/archsurg.1992.01420120070013. PMID: 1365690

Prognosis

Perianal Paget disease treated with wide excision and thigh skin flap reconstruction: a case report and review of literature.
Shen K, Luo H, Hu J, Xie Z
Medicine (Baltimore) 2018 Jul;97(30):e11638. doi: 10.1097/MD.0000000000011638. PMID: 30045309Free PMC Article
Staged marginal contoured and central excision technique in the surgical management of perianal Paget's disease.
Möller MG, Lugo-Baruqui JA, Milikowski C, Salgado CJ
Am J Surg 2014 Apr;207(4):485-92. Epub 2013 Sep 20 doi: 10.1016/j.amjsurg.2013.04.005. PMID: 24055144
Management of less common tumors of the colon, rectum, and anus.
Cuffy M, Abir F, Longo WE
Clin Colorectal Cancer 2006 Jan;5(5):327-37. doi: 10.3816/CCC.2006.n.002. PMID: 16512991
Perianal Paget's disease: case report and review of the literature.
Delaunoit T, Neczyporenko F, Duttmann R, Deprez C, da Costa PM, de Koster E
Acta Gastroenterol Belg 2004 Apr-Jun;67(2):228-31. PMID: 15285581
Paget's disease of the anal margin.
Jensen SL, Sjølin KE, Shokouh-Amiri MH, Hagen K, Harling H
Br J Surg 1988 Nov;75(11):1089-92. doi: 10.1002/bjs.1800751113. PMID: 2850073

Clinical prediction guides

Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy.
Dos Santos JS, Bonafé GA, Pereira JA, Kanno DT, Martinez CAR, Ortega MM
BMC Cancer 2018 Sep 25;18(1):921. doi: 10.1186/s12885-018-4815-6. PMID: 30253739Free PMC Article
Staged excision and split-thickness skin graft for circumferential perianal Paget's disease.
Lam DT, Batista O, Weiss EG, Nogueras JJ, Wexner SD
Dis Colon Rectum 2001 Jun;44(6):868-70. doi: 10.1007/BF02234711. PMID: 11391150

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