Download PDF
CC BY 4.0 · Journal of Coloproctology 2024; 44(02): e141-e144
DOI: 10.1055/s-0044-1782156
Case Report

Perianal Paget's Disease Metachronically Associated with Adenocarcinoma

Camila Vieira Chiquetti 
1   Hospital Regional do Mato Grosso do Sul (HRMS), MS, Brazil
,
Victor Hugo de Araújo Gonçalves
2   Medical Student, Universidade Estadual do Mato Grosso do Sul (UEMS), MS, Brazil
,
Leonardo Carnielli Uliana
2   Medical Student, Universidade Estadual do Mato Grosso do Sul (UEMS), MS, Brazil
,
Carlos Otávio da Silva Ribeiro
3   Member of the Society Brazilian Journal of Coloproctology
,
Odinilson Almeida Fonseca
3   Member of the Society Brazilian Journal of Coloproctology
,
Carlos Henrique Marques dos Santos
3   Member of the Society Brazilian Journal of Coloproctology
,
Luiz Carlos Takita
4   Faculdade de Medicina (FAMED), Universidade Federal do Mato Grosso do Sul (UFMS), MS, Brazil
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Abstract

Introduction This article describes an unusual case of perianal Paget's Disease in a 42-year-old man, highlighting distinct characteristics about more typical cases.

Case report 42-year-old male patient with persistent perianal lesion, recurrent abscesses, and discomfort. Magnetic resonance imaging indicated a possible fistulous path. Biopsy revealed secondary anal Paget's disease and adenocarcinoma. Chemotherapy and radiotherapy treatment demonstrated a positive response.

Discussion Perianal Paget's Disease is a rare condition of the anal canal that is part of the Extramammary Paget's Disease (EMPD) group. Symptoms include itching, pain, perianal bleeding, and skin lesions. Diagnosis can be challenging and usually involves performing a histopathological biopsy associated with an immunohistochemical study. Treatment varies and may involve surgery, radiotherapy, and chemotherapy. Due to the rarity of the disease, the best therapeutic approach is not yet well defined, and follow-up is essential due to the risk of recurrence.

Conclusion Perianal Paget's Disease is rare and requires differentiation from other conditions. Early detection is essential to prevent malignancy.

Keywords

Extramammary Paget's Disease - rectal fistula - Diseases of the Anus - anal channel


Publication History

Received: 25 October 2023

Accepted: 07 February 2024

Article published online:
06 June 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

Thieme Revinter Publicações Ltda.
Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil

 

  • References

  • 1 Navajas Hernández P, Valdés Delgado T, Machuca Aguado J, González-Cámpora R, Argüelles Arias F. Perianal Paget's disease. Rev Esp Enferm Dig 2022; ••• : Epub ahead of print DOI: 10.17235/reed.2022.9304/2022. PubMed
    CrossrefPubMedGoogle Scholar
  • 2 Merichal Resina M, Cerdan Santacruz C, Sierra Grañón E, Tarragona Foradada JA, Olsina Kissler JJ. Enfermedad de Paget perianal. Cir Esp 2018; DOI: 10.1016/j.ciresp.2018.08.00.
    CrossrefGoogle Scholar
  • 3 Rudnicki Y, Stapleton SM, Batra R, Gan T, Mathis KL, Kelley SR. Perianal Paget's-an aggressive disease. Colorectal Dis 2023; 25: 1213-1221 Epub ahead of print DOI: 10.1111/codi.16549. PubMed
    CrossrefPubMedGoogle Scholar
  • 4 Simonds RM, Segal RJ, Sharma A. Extramammary Paget's disease: a review of the literature. Int J Dermatol 2019; 58 (08) 871-879 DOI: 10.1111/ijd.14328.
    CrossrefPubMedGoogle Scholar
  • 5 Thompson HM, Kim JK. Perianal Paget's Disease. Dis Colon Rectum 2021; 64 (05) 511-515 DOI: 10.1097/DCR.0000000000002000.
    CrossrefPubMedGoogle Scholar
  • 6 St Claire K, Hoover A, Ashack K, Khachemoune A. Extramammary Paget disease. Dermatol Online J 2019; 25 (04) 13030/qt7qg8g292
    Google Scholar
  • 7 Ventura S, Carvalho A, Rodrigues C, Domingues Â, Cancela E, Silva A. Perianal Paget's disease - A diagnosis to consider. Rev Esp Enferm Dig 2022; ••• : Epub ahead of print DOI: 10.17235/reed.2022.9224/2022. PubMed
    CrossrefPubMedGoogle Scholar
  • 8 Hutchings D, Windon A, Assarzadegan N, Salimian KJ, Voltaggio L, Montgomery EA. Perianal Paget's disease as spread from non-invasive colorectal adenomas. Histopathology 2021; 78 (02) 276-280 DOI: 10.1111/his.14218.
    CrossrefPubMedGoogle Scholar
  • 9 Kim BJ, Park SK, Chang H. The effectiveness of mapping biopsy in patients with extramammary Paget's disease. Arch Plast Surg 2014; 41 (06) 753-758
    Article in Thieme ConnectPubMedGoogle Scholar
  • 10 Perez DR, Trakarnsanga A, Shia J. et al. Management and outcome of perianal Paget's disease: a 6-decade institutional experience. Dis Colon Rectum 2014; 57 (06) 747-751
    CrossrefPubMedGoogle Scholar
  • 11 Vereecken P, Awada A, Ghanem G. et al. A therapeutic approach to perianal extramammary Paget's disease: topical imiquimod can be useful to prevent or defer surgery. Med Sci Monit 2007; 13 (06) CS75-CS77
    PubMedGoogle Scholar
  • 12 Ho SA, Aw DC. Extramammary Paget's disease treated with topical imiquimod 5% cream. Dermatol Ther 2010; 23 (04) 423-427
    CrossrefPubMedGoogle Scholar
  • 13 Mann J, Lavaf A, Tejwani A, Ross P, Ashamalla H. Perianal Paget disease treated definitively with radiotherapy. Curr Oncol 2012; 19 (06) e496-e500 DOI: 10.3747/co.19.1144.
    CrossrefPubMedGoogle Scholar
  • 14 Brown RS, Lankester KJ, McCormack M, Power DA, Spittle MF. Radiotherapy for perianal Paget's disease. Clin Oncol (R Coll Radiol) 2002; 14 (04) 272-284
    CrossrefPubMedGoogle Scholar
  • 15 Besa P, Rich TA, Delclos L, Edwards CL, Ota DM, Wharton JT. Extramammary Paget's disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys 1992; 24 (01) 73-78
    CrossrefPubMedGoogle Scholar
  • 16 Möller MG, Lugo-Baruqui JA, Milikowski C, Salgado CJ. Staged marginal contoured and central excision technique in the surgical management of perianal Paget's disease. Am J Surg 2014; 207 (04) 485-492
    CrossrefPubMedGoogle Scholar