Diseases of the Colon & Rectum

, Volume 33, Issue 6, pp 502–507 | Cite as

Perianal Paget's disease

Classification and review of management: Report of two cases
  • William P. Shutze
  • John J. Gleysteen
Case Reports


Fewer than 100 cases of Paget's disease located in the perianal skin have been reported since the extramammary location was first described in 1893. Two patients seen and treated in the authors' institutions with disease limited to the epidermis and its adnexae are described to illustrate the usual presentation and pathobiology of the disease. A staging classification based on the disease pathology has been developed from the cases reported in the literature and correlated with appropriate surgical treatment. Stage I disease treated with wide local excision promises unlimited survival, whereas the prognosis worsens for Stage II, with synchronous localized malignancies, and for Stages III and IV, with regional and distant metastatic disease, respectively.

Key words

Paget's disease, extramammary Perianal gland neoplasms 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Berardi B, Lee S, Chen HP: Perianal extramammary Paget's disease. Surg Gynecol Obstet 1988;167:359–66.PubMedGoogle Scholar
  2. 2.
    Beck DE, Fazio VW. Perianal Paget's disease. Dis Colon Rectum 1987;30:263–66.PubMedGoogle Scholar
  3. 3.
    Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Barth Hosp Rep 1874;10:87–9.Google Scholar
  4. 4.
    Darier J, Couilland P. Sur un cas de maladie de Paget de la region perineo-anale et scrotale. Ann Dermatol Syph 1893;4:25–31.Google Scholar
  5. 5.
    Nagle RB, Lucas DO, McDaniel KM, Clark VA, Schmazel GM. Paget's cells, new evidence linking mammary and extramammary Paget cells to a common cell phenotype. Am J Clin Pathol 1985;83:431–8.PubMedGoogle Scholar
  6. 6.
    Kuo T, Chan HL, Hsueh S. Clear cell papulosis of the skin: a new entity with histogenetic implication for cutaneous Paget's disease. Am J Surg Pathol 1987;11:827–34.PubMedGoogle Scholar
  7. 7.
    Kariniemi AL, Forsman L, Wahlstrum T, Verterinen E, Anderson L. Expression of differentiation antigens in mammary and extramammary Paget's disease. Br J Dermatol 1984;110:203–10.PubMedGoogle Scholar
  8. 8.
    Nadji M, Morales AR, Girtanner RT, Ziegls-Weisman J, Penneys NS. Paget's disease of the skin: a unifying concept in histogenesis. Cancer 1982;50:2203–6.PubMedGoogle Scholar
  9. 9.
    Ferenzy A, Richart RM. Ultrastructure of perianal Paget's disease. Cancer 1972;29:1141–9.PubMedGoogle Scholar
  10. 10.
    Helwig EB, Graham JH. Anogenital (extramammary) Paget's disease: a clinicopathologic study. Cancer 1963;16:387–403.PubMedGoogle Scholar
  11. 11.
    Jones RE, Austin C, Ackerman AB. Extramammary Paget's disease: a critical reexamination. Am J Dermatopathol 1979;1:101–31.PubMedGoogle Scholar
  12. 12.
    Rosen L, Amazon K, Frank B. Bowen's disease, Paget's disease, and malignant melanoma in situ. South Med J 1986;79:410–13.PubMedGoogle Scholar
  13. 13.
    Ordonez NG, Await H, MacKay B. Mammary and extramammary Paget's disease: an immunocytochemical and ultrastructural study. Cancer 1987;59:1173–83.PubMedGoogle Scholar
  14. 14.
    Mazoujian G, Pinkus G, Haagensen ED. Extramammary Paget's disease—evidence for an apocrine origin: an immunoperoxidase study of gross cystic disease fluid protein-15, carcinoembryonic antigen and keratin proteins. Am J Surg Pathol 1984;8:43–50.PubMedGoogle Scholar
  15. 15.
    Balducei L, Athar M, Smith GF, Khansur T, McKenzie D, Crawford ED. Metastatic extramammary Paget's disease: dramatic response to combined modality treatment. J Surg Oncol 1988;38:38–44.PubMedGoogle Scholar
  16. 16.
    Secco GB, Lapertosa G, Sertoli MR, Scarpati D, Riboli EB. Perianal Paget's disease: case report of an elderly patient treated with polychemotherapy and radiotherapy. Tumori 1984;70:381–3.PubMedGoogle Scholar
  17. 17.
    Archer CB, Louback JB, MacDonald DM. Spontaneous regression of perianal extramammary Paget's disease after partial surgical excision. Arch Dermatol 1987;123:379–82.PubMedGoogle Scholar
  18. 18.
    Sharkey FE, Clarke RL, Gray GF. Perianal Paget's disease: report of two cases. Dis Colon Rectum 1975;18:245–8.PubMedGoogle Scholar
  19. 19.
    William SL, Rogers LW, Quan SH. Perianal Paget's disease: report of seven cases. Dis Colon Rectum 1976;19:30–40.PubMedGoogle Scholar
  20. 20.
    Pitman GH, McCarthy JG, Perzin KH, Herter FP. Extramammary Paget's disease. Plast Reconstr Surg 1982;69:238–44.PubMedGoogle Scholar
  21. 21.
    Kavlie H, Stevenson JK, Gould VE. Perianal Paget's disease. Am Surg 1971;37:485–9.PubMedGoogle Scholar
  22. 22.
    Oster MW, Magun A, Herter FP, Wolff M. Colorectal carcinoma 15 years after the diagnosis of perianal Pagets. J Surg Oncol 1979;12:379–84.PubMedGoogle Scholar
  23. 23.
    Chandra JJ. Extramammary Paget's disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol 1985;13:1009–14.PubMedGoogle Scholar
  24. 24.
    James LP, Worsham GF, Hoskins WJ, Belcik R. Apocrine adenocarcinoma of the vulva with associated Paget's disease, diagnosis of lymph node metastases by fine needle aspiration biopsy. Acta Cytol 1984;28:178–84.PubMedGoogle Scholar
  25. 25.
    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.PubMedGoogle Scholar
  26. 26.
    Relias A, Maissa S: Maladic de Paget a la region peri-anale. Societe de Dermatologie et de Syphiligraphie 1957;64:368.Google Scholar
  27. 27.
    Bocian JJ, Tuschka O. Paget's disease of the anus. Am J Surg 1957;93:140–2.PubMedGoogle Scholar
  28. 28.
    Fisher GR, Beyer FD. Extramammary Paget's disease. Am J Surg 1957;94:493–8.PubMedGoogle Scholar
  29. 29.
    Nelson TF. Perianal Paget's disease. Dis Colon Rectum 1960;3:135–7.PubMedGoogle Scholar
  30. 30.
    Straus R. Extramammary Paget's disease of the anus. Am J Proctol 1964;15:36–47.PubMedGoogle Scholar
  31. 31.
    Rabson AS, VanScott EJ, Smith RB. Carcinoma of the anorectal junction with “extramammary Paget's disease”. Arch Pathol 1958;65:432–7.Google Scholar
  32. 32.
    Hutcheson JB, Gordon JB, Fuqua WN JR. “Extramammary Paget's disease” of the anorectal junction. Arch Pathol 1960;69:728–32.PubMedGoogle Scholar
  33. 33.
    Green WW, Epstein FW. Perianal extramammary Paget's disease. Surgery 1950;28:44–7.PubMedGoogle Scholar
  34. 34.
    O'Grady JF, Hewitt SR. Extramammary Paget's disease of the anus. IJ Med Sci 1968;1:291–4.Google Scholar
  35. 35.
    Burt CA. Thick split-skin graft for anal stenosis, Paget's disease, condyloma acuminata, pruritus ani, and other anorectal conditions: report of four cases. Dis Colon Rectum 1961;4:319–26.PubMedGoogle Scholar
  36. 36.
    Zoon, JJ, Gelpke AS. Findings in a case of extra-mammary Paget's disease. Dermatologica 1950;101:153–7.PubMedGoogle Scholar
  37. 37.
    Pinkus H, Gould SE. Extramammary Paget's disease and intraepidermal carcinoma. Arch Dermat Syph 1939;39:479–502.Google Scholar
  38. 38.
    Anzt L, Keen O. Die Paget disease mit besonserer berucksichtigung ihrer pathogenese. Arch J Dermat Syph 1925:148:284–312.Google Scholar
  39. 39.
    Dockerty MB, Pratt JH. Extramammary Paget's disease: a report of four cases in which certain features of histogenesis were exhibited. Cancer 1952;5:1161–9.PubMedGoogle Scholar
  40. 40.
    Yuell JH, Price WG. Paget's disease of the perianal skin with associated adenocarcinoma. Arch Dermatol 1960;82:986–91.PubMedGoogle Scholar
  41. 41.
    Pearson LR, McArt BA. Paget's disease of the anus: case report. Am J Surg 1953;86:551–2.PubMedGoogle Scholar
  42. 42.
    Lock MA, Parka A, Katz DR, Thomson JP: Perianal Paget's disease. Postgrad Med J 1977;53:768–72.PubMedGoogle Scholar
  43. 43.
    Merot Y, Mazoujian G, Pinkus G, Momtaz TK, Murphy GF. Extramammary Paget's disease of the perianal and perineal regions: evidence of apocrine derivation. Arch Dermatol 1985;121:750–2.PubMedGoogle Scholar
  44. 44.
    Giltman LI, Osborne PT, Coleman SA, Uthman EO. Paget's disease of the anal mucosa in association with carcinoma demonstrating mucoepidermal features. J Surg Oncol 1985;28:277–80.PubMedGoogle Scholar
  45. 45.
    Grodsky L. Rare nonkerotonizing malignancies of anal region: pathologic features. Arch Surg 1965;90:216–21.PubMedGoogle Scholar
  46. 46.
    Grow JR, Kshirsagar V, Tolentino M, Gramling J, Schutte AG. Extramammary perianal Paget's disease: report of a case. Dis Colon Rectum 1977;20:436–42.PubMedGoogle Scholar
  47. 47.
    Linder JH, Myers RT. Perianal Paget's disease. Am Surg 1970;36:342–5.PubMedGoogle Scholar
  48. 48.
    Weiber H, Ekelund G, Lindstrom C. Perianal Paget's disease. Acta Chir Scand 1987;153:687–90.PubMedGoogle Scholar
  49. 49.
    Subbuswamy SG, Ribeiro BF. Perianal Paget's disease associated with cloacogenic carcinoma: report of a case. Dis Colon Rectum 1981;24:535–8.PubMedGoogle Scholar
  50. 50.
    Langsford G, Kermode D. Perianal Paget's disease: a case report. Aust NZ J Surg 1980;50:181–3.Google Scholar
  51. 51.
    Tjandra J. Perianal Paget's disease: report of three cases. Dis Colon Rectum 1988;31:462–6.PubMedGoogle Scholar
  52. 52.
    Jackson BR. Extramammary Paget's disease and anaplastic basaloid small-cell carcinoma of the anus: report of a case. Dis Colon Rectum 1975;18:339–45.PubMedGoogle Scholar
  53. 53.
    Grodsky L. Extramammary Paget's disease of the perianal region. Dis Colon Rectum 1960;3:502–10.PubMedGoogle Scholar
  54. 54.
    Becker GL, Bacon HE. Extramammary Paget's disease—epidermotrophic carcinoma of the anal region: report of a case and review of the literature. Dis Colon Rectum 1963;6:449–53.Google Scholar

Copyright information

© American Society of Colon and Rectal Surgeons 1990

Authors and Affiliations

  • William P. Shutze
    • 1
    • 2
  • John J. Gleysteen
    • 1
    • 2
  1. 1.Department of SurgeryUniversity of Alabama at BirminghamUK
  2. 2.Surgical ServiceVeterans Affairs Medical CenterBirmingham

Personalised recommendations