U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Anal canal Paget disease

MedGen UID:
231062
•Concept ID:
C1332261
•
Neoplastic Process
Synonyms: anal canal mammary Paget's disease; Anal Canal Paget Disease; anal canal Paget disease; Anal Canal Paget's Disease; anal canal Paget's disease; Paget disease of anal canal; Paget Disease of the Anal Canal; Paget disease of the anal canal; Paget's Disease of Anal Canal; Paget's disease of anal canal; Paget's Disease of the Anal Canal; Paget's disease of the anal canal; Pagets disease of anal canal
SNOMED CT: Pagets disease of anal canal (236811000119101); Paget disease of anal canal (236811000119101)
 
Monarch Initiative: MONDO:0004468

Definition

Paget disease involving the squamous epithelium of the anal canal. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Spectrum of Changes in Anogenital Mammary-like Glands in Primary Extramammary (Anogenital) Paget Disease and Their Possible Role in the Pathogenesis of the Disease.
Konstantinova AM, Spagnolo DV, Stewart CJR, Kacerovska D, Shelekhova KV, Plaza JA, Suster S, Bouda J, Kyrpychova L, Michal M, Belousova IE, Kerl K, Kazakov DV
Am J Surg Pathol 2017 Aug;41(8):1053-1058. doi: 10.1097/PAS.0000000000000857. PMID: 28614205
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
Treatment of intractable pruritus ani.
Eusebio EB, Graham J, Mody N
Dis Colon Rectum 1990 Sep;33(9):770-2. doi: 10.1007/BF02052324. PMID: 2390913

Recent clinical studies

Etiology

Goblet cell adenocarcinoma of the anal canal with perianal Paget disease: A rare case report with literature review.
Li M, Yao X
Medicine (Baltimore) 2023 Apr 21;102(16):e33598. doi: 10.1097/MD.0000000000033598. PMID: 37083794Free PMC Article
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 secondary to pagetoid spread of mucinous adenocarcinoma of the anal canal.
Matos-Pires E, Oliveira M, João A, Mendes-Bastos P
Eur J Dermatol 2018 Apr 1;28(2):270-271. doi: 10.1684/ejd.2018.3237. PMID: 29400297
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
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

Diagnosis

Anal and Perianal Preneoplastic Lesions.
Loughrey MB, Shepherd NA
Gastroenterol Clin North Am 2024 Mar;53(1):201-220. Epub 2023 Oct 16 doi: 10.1016/j.gtc.2023.09.007. PMID: 38280748
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
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 Goblet Cell Carcinoid With Pagetoid Spread.
Grunes D, Kim MK, Chessin DB, Yaeger RD, Ward SC
Int J Surg Pathol 2019 Oct;27(7):788-791. Epub 2019 May 13 doi: 10.1177/1066896919845478. PMID: 31079516Free PMC Article
Anogenital cutaneous lesions.
Kierland RR
JAMA 1968 Jan 15;203(3):213-8. PMID: 4294553

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
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
A therapeutic approach to perianal extramammary Paget's disease: topical imiquimod can be useful to prevent or defer surgery.
Vereecken P, Awada A, Ghanem G, Marques da Costa C, Larsimont D, Simoens C, Mendes da Costa P, Hendlisz A
Med Sci Monit 2007 Jun;13(6):CS75-7. PMID: 17534239
Treatment of intractable pruritus ani.
Eusebio EB, Graham J, Mody N
Dis Colon Rectum 1990 Sep;33(9):770-2. doi: 10.1007/BF02052324. PMID: 2390913
A review of lower genital intraepithelial neoplasia and the use of topical 5-fluorouracil.
Sillman FH, Sedlis A, Boyce JG
Obstet Gynecol Surv 1985 Apr;40(4):190-220. doi: 10.1097/00006254-198504000-00002. PMID: 2984615

Prognosis

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
Perianal Goblet Cell Carcinoid With Pagetoid Spread.
Grunes D, Kim MK, Chessin DB, Yaeger RD, Ward SC
Int J Surg Pathol 2019 Oct;27(7):788-791. Epub 2019 May 13 doi: 10.1177/1066896919845478. PMID: 31079516Free 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
Perianal Paget disease secondary to pagetoid spread of mucinous adenocarcinoma of the anal canal.
Matos-Pires E, Oliveira M, João A, Mendes-Bastos P
Eur J Dermatol 2018 Apr 1;28(2):270-271. doi: 10.1684/ejd.2018.3237. PMID: 29400297
Double Rarities, Double Challenges: Extra-Mammary Paget's Disease and Anal Adenocarcinoma.
Gholami S, Wood LS, Bingham D, Kin C
Dig Dis Sci 2016 Apr;61(4):996-9. Epub 2015 Aug 2 doi: 10.1007/s10620-015-3819-1. PMID: 26233548

Clinical prediction guides

Perianal Paget's disease complicated with lung adenocarcinoma and anal canal carcinoma: A case report and literature review.
Zhou L, Yang M, Wang T, Zhang J
Zhong Nan Da Xue Xue Bao Yi Xue Ban 2023 Aug 28;48(8):1274-1280. doi: 10.11817/j.issn.1672-7347.2023.220617. PMID: 37875369Free PMC Article
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
Characteristic Clinicopathological Features of Secondary Extramammary Paget Disease With Underlying Anorectal Adenocarcinoma: Evenly Circumferential Perianal Distribution, Fibroepithelioma of Pinkus-like Changes, and Subepidermal Mucin Deposits Without Invasive Tumor Cells.
Sasaki Y, Goto K, Sugino T, Satake K, Yoshikawa S, Kiyohara Y
Am J Dermatopathol 2021 Oct 1;43(10):721-726. doi: 10.1097/DAD.0000000000001877. PMID: 33395042
The use of cytokeratins 7 and 20 in the diagnosis of primary and secondary extramammary Paget's disease.
Ohnishi T, Watanabe S
Br J Dermatol 2000 Feb;142(2):243-7. doi: 10.1046/j.1365-2133.2000.03291.x. PMID: 10730755
Treatment of intractable pruritus ani.
Eusebio EB, Graham J, Mody N
Dis Colon Rectum 1990 Sep;33(9):770-2. doi: 10.1007/BF02052324. PMID: 2390913

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...