Diseases of the Colon & Rectum

, Volume 51, Issue 12, pp 1842–1845 | Cite as

Perianal Paget’s Disease Treated with Wide Excision and Gluteal Skin Flap Reconstruction: Report of a Case and Review of the Literature

  • Wolfgang B. GaertnerEmail author
  • Gonzalo F. Hagerman
  • Stanley M. Goldberg
  • Charles O. FinneIII
Multimedia Article



Paget’s disease of the anus is a rare perianal disorder. The condition is often associated with underlying invasive carcinoma and the prognosis is poor when underlying adenocarcinoma is present.


We report the case of a 72-year-old woman suffering from a two-year history of perianal itching, bleeding, and irritation. Perianal Paget’s disease was confirmed by histopathologic demonstration of Paget’s cells from a biopsy. Synchronous malignancy and metastasis were excluded.


A first-stage operation consisted of a wide excisional biopsy with frozen section margin assessment. Permanent sections confirmed absence of invasive carcinoma and clear margins. Four days after wide excision, bilateral gluteal skin flap reconstruction was performed with a temporary diverting colostomy. No adjuvant therapy was necessary, and after one-year follow-up, the patient remains disease free.


Perianal Paget’s disease continues to pose problems in diagnosis and treatment. Our case report of perianal Paget’s disease shows one method of management for advanced non invasive disease.

Key words

Perianal Paget’s disease Treatment Surgery 

Supplementary material


(MPG 169 KB)


  1. 1.
    Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Barth Hosp Rep 1874;10:87–9.Google Scholar
  2. 2.
    Darier J, Coulillad P. Sur un cas de maladie de Paget de la region perinea-anale et scrotale. Ann de Dermatol and de Syph 1893;4:25–31.Google Scholar
  3. 3.
    Berardi RS, Lee S, Chen HP. Perianal extramammary Paget’s disease. Surg Gynecol Obstet 1988;167:359–65.PubMedGoogle Scholar
  4. 4.
    Thirlby RC, Hammer CJ, Galagan KA, Travaglini JJ, Picazzi VJ. Perianal Paget’s disease: successful treatment with combined chemoradiotherapy: report of a case. Dis Colon Rectum 1990;33:150–2.PubMedCrossRefGoogle Scholar
  5. 5.
    Tjandra J. Perianal Paget’s disease: report of three cases. Dis Colon Rectum 1988;31:462–6.PubMedCrossRefGoogle Scholar
  6. 6.
    Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM. Paget’s disease of the perianal region—an aggressive disease? Dis Colon Rectum 1997;40:1187–94.PubMedCrossRefGoogle Scholar
  7. 7.
    Butler JD, Hershman MJ, Wilson CA, Bryson JR. Perianal Paget’s disease. J R Soc Med 1997;90:688–9.PubMedGoogle Scholar
  8. 8.
    Lock MR, Katz DR, Parks A, Thomson JP. Perianal Paget’s disease. Postgrad Med J 1977;53:768–72.PubMedCrossRefGoogle Scholar
  9. 9.
    Zollo JD, Zeitouni NC. The Roswell Park cancer institute experience with extramammary Paget’s disease. Br J Dermatol 2000;142:59–65.PubMedCrossRefGoogle Scholar
  10. 10.
    Marchesa P, Fazio VW, Oliart S, Goldblum JR, Lavery IC, Milson JW. Long-term outcome of patients with perianal Paget’s disease. Ann Surg Oncol 1997;4:475–80.PubMedCrossRefGoogle Scholar
  11. 11.
    Chanda JJ. Extramammary Paget’s disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol 1985;13:1009–14.PubMedCrossRefGoogle Scholar
  12. 12.
    Goldman S, Ihre T, Logerstedt U, Svensson C. Perianal Paget’s disease: report of five cases. Int J Colorectal Dis 1992;7:167–9.PubMedCrossRefGoogle Scholar
  13. 13.
    Jones RE Jr, Austin C, Ackerman AB. Extramammary Paget’s disease. A critical reexamination. Am J Dermatopathol 1979;1:101–32.PubMedCrossRefGoogle Scholar
  14. 14.
    Wood WS, Culling CF. Perianal Paget’s disease: histochemical differentiation utilizing the borohydride-KOH-PAS reaction. Arch Pathol 1975;99:442–5.PubMedGoogle Scholar
  15. 15.
    Arminski TC, Pollard RJ. Paget’s disease of the anus secondary to a malignant papillary adenoma of the rectum. Dis Colon Rectum 1973;16:46–55.PubMedCrossRefGoogle Scholar
  16. 16.
    Lloyd J, Flanagan AM. Mammary and extramammary Paget’s disease. J Clin Pathol 2000;53:742–9.PubMedCrossRefGoogle Scholar
  17. 17.
    Armitage NC, Jass JR, Richman PI, Thomson JP, Phillips RK. Paget’s disease of the anus: a clinicopathological study. Br J Surg 1989;76:60–3.PubMedCrossRefGoogle Scholar
  18. 18.
    Goldblum JR, Hart WR. Perianal Paget’s disease. A histologic and immunohistochemical study with and without associated rectal adenocarcinoma. Am J Surg Pathol 1998;22:170–9.PubMedCrossRefGoogle Scholar
  19. 19.
    Nowak MA, Guerriere-Kovach P, Pathan A, Campbell TE, Deppisch LM. Perianal Paget’s disease. Distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression. Arch Pathol Lab Med 1998;122:1077–81.PubMedGoogle Scholar
  20. 20.
    Beck DE, Fazio VW. Perianal Paget’s disease. Dis Colon Rectum 1987;30:263–6.PubMedCrossRefGoogle Scholar
  21. 21.
    McCarter MD, Quan SH, Busam K, Paty PP, Wong D, Guillem JG. Long-term outcome of perianal Paget’s disease. Dis Colon Rectum 2003;46:612–6.PubMedCrossRefGoogle Scholar
  22. 22.
    Mehta NJ, Torno R, Sorra T. Extramammary Paget’s disease. Southern Med J 2000;93:713–6.Google Scholar
  23. 23.
    Burrows NP, Jones DH, Hudson PM, Pye RJ. Treatment of extramammary Paget’s disease by radiotherapy. Br J Dermatol 1995;132:970–2.PubMedGoogle Scholar
  24. 24.
    Shieh S, Dee AS, Cheney RT, Frawley NP, Zeitouni NC, Oseroff AR. Photodynamic therapy for the treatment of extramammary Paget’s disease. Br J Dermatol 2002;146:1000–5.PubMedCrossRefGoogle Scholar
  25. 25.
    Moreno-Arias GA, Conill C, Castells-Mas A, Arenas M, Grimalt R. Radiotherapy for genital extramammary Paget’s disease in situ. Dermtol Surg 2001;27:587–90.CrossRefGoogle Scholar
  26. 26.
    Brierly JD, Stockdale AD. Radiotherapy: an effective treatment for extramammary Paget’s disease. J Clin Oncol 1991;3:3–5.CrossRefGoogle Scholar
  27. 27.
    Zamponga JC, Flowers FP, Roth WI, Hassenein AM. Treatment of primary limited cutaneous extramammary Paget’s disease with topical imiquimod monotherapy: two case reports. J Am Acad Dermatol 2002;47:S229–35.CrossRefGoogle Scholar
  28. 28.
    Murakami K, Tanimura H, Ishimoto K, et al. Reconstruction with bilateral gluteus maximus myocutaneous rotation flap after wide local excision for perianal Paget’s disease. Dis Colon Rectum 1996;39:227–31.PubMedCrossRefGoogle Scholar
  29. 29.
    Brooke BR, Schoetz DJ, Coller JA. Skin grafts for circumferential coverage of Perianal wounds. Surg Clin North Am 1985;65:365–71.Google Scholar
  30. 30.
    Lam DT, Batista O, Weiss EG, et al. Staged excision and split thickness skin graft for circumferential perianal Paget’s disease. Dis Colon Rectum 2001;44:868–70.PubMedCrossRefGoogle Scholar
  31. 31.
    Shutze WP, Gleysteen JJ. Perianal Paget’s disease. Classification and review of management: report of two cases. Dis Colon Rectum 1990;33:502–7.PubMedCrossRefGoogle Scholar
  32. 32.
    Jensen SL, Sjølin KE, Shokouh-Amiri MH, Hagen K, Harling H. Paget’s disease of the anal margin. Br J Surg 1988;75:1089–92.PubMedCrossRefGoogle Scholar
  33. 33.
    Pierie JP, Choudry U, Muzikansky A, Finkelstein DM, Ott MJ. Prognosis and management of extramammary Paget’s disease and the association with secondary malignancies. J Am Coll Surg 2003;196:45–50.PubMedCrossRefGoogle Scholar
  34. 34.
    Williams SL, Rogers LW, Quan SH. Perianal Paget’s disease: report of seven cases. Dis Colon Rectum 1976;19:30–40.PubMedCrossRefGoogle Scholar

Copyright information

© The American Society of Colon and Rectal Surgeons 2008

Authors and Affiliations

  • Wolfgang B. Gaertner
    • 1
    Email author
  • Gonzalo F. Hagerman
    • 1
  • Stanley M. Goldberg
    • 1
  • Charles O. FinneIII
    • 1
  1. 1.Division of Colon and Rectal Surgery, Department of SurgeryUniversity of MinnesotaMinneapolisUSA

Personalised recommendations