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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'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
Malignancies of the anal canal.
Lintzeris I, Agrogianni X, Prigouris P, Lintzeri A, Giovannakis K, Argyrakos T, Rodoyianni I, Prigouris S
Tech Coloproctol 2011 Oct;15 Suppl 1:S87-9. doi: 10.1007/s10151-011-0753-x. PMID: 21887557
Carcinoma of the anus.
Beahrs OH, Wilson SM
Ann Surg 1976 Oct;184(4):422-8. doi: 10.1097/00000658-197610000-00004. PMID: 189707Free PMC Article

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 Paget's disease as spread from non-invasive colorectal adenomas.
Hutchings D, Windon A, Assarzadegan N, Salimian KJ, Voltaggio L, Montgomery EA
Histopathology 2021 Jan;78(2):276-280. Epub 2020 Sep 23 doi: 10.1111/his.14218. PMID: 32705713Free 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

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
Anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease, successfully treated with modified FOLFOX6: a case report.
Yamaura M, Yamada T, Watanabe R, Kawai H, Hirose S, Tajima H, Sato M, Uchida Y, Suganuma D, Yamamoto Y, Moriwaki T, Hyodo I
BMC Cancer 2018 Nov 20;18(1):1142. doi: 10.1186/s12885-018-5084-0. PMID: 30458812Free 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
Extramammary Paget's disease of the perianal region.
Tulchinsky H, Zmora O, Brazowski E, Goldman G, Rabau M
Colorectal Dis 2004 May;6(3):206-9. doi: 10.1111/j.1463-1318.2004.00630.x. PMID: 15109389
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

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 Paget's disease as spread from non-invasive colorectal adenomas.
Hutchings D, Windon A, Assarzadegan N, Salimian KJ, Voltaggio L, Montgomery EA
Histopathology 2021 Jan;78(2):276-280. Epub 2020 Sep 23 doi: 10.1111/his.14218. PMID: 32705713Free PMC Article
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
Malignancies of the anal canal.
Lintzeris I, Agrogianni X, Prigouris P, Lintzeri A, Giovannakis K, Argyrakos T, Rodoyianni I, Prigouris S
Tech Coloproctol 2011 Oct;15 Suppl 1:S87-9. doi: 10.1007/s10151-011-0753-x. PMID: 21887557
Carcinoma of the anus.
Beahrs OH, Wilson SM
Ann Surg 1976 Oct;184(4):422-8. doi: 10.1097/00000658-197610000-00004. PMID: 189707Free PMC Article

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
Anal neoplasia and its precursors: facts and controversies.
Fenger C
Semin Diagn Pathol 1991 Aug;8(3):190-201. PMID: 1656504
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

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