Skip to main content
SpringerLink
Log in

Long-term outcome of patients with perianal Paget's disease

  • Original Articles
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background: Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma with a significant rate of recurrence after treatment and high risk of progression to an invasive cancer.

Patients and Methods: Fourteen patients with a mean follow-up longer than 5 years were studied to determine the outcome after surgical treatment. The immunohistochemical accumulation of p53 protein also was assessed in tissue specimens to evaluate its prognostic role in patients with PPD.

Results: Four patients were excluded because of progression to invasive malignancy at the time of diagnosis. Two patients underwent local excision (LE) with macroscopic clearance of the surgical margins; the remaining eight patients underwent wide local excision (WLE), i.e., >1 cm microscopic clearance of the surgical margins. The actuarial 8-year recurrence rate for patients treated with LE and WLE was 100% and 50% (SE=17.7), respectively. Progression to invasive carcinoma occurred after a median time of 56 months (range 23–72) in two patients treated with LE and in one of eight patients treated with WLE. All four patients with recurrence after WLE were successfully treated (no further recurrence) with a second WLE. Actuarial 8-year survival was 0% in the LE group and 40% (SE=21.9) in the WLE group. There was no p53 protein accumulation in any of the ten patients with PPD.

Conclusions: Survival of patients with PPD treated by WLE was higher than that for those treated with LE. Thus, wide local excision is recommended over limited local excision as a preferred treatment for PPD. Follow-up longer than 5 years seems to be indicated because of the risk of late progression to invasive cancer. When PPD does recur, a second WLE may be curative. The absence of accumulated p53 protein suggests that this marker may not have a prognostic role in PPD.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Guarnev J, Cohen C, Derose PB. Histogenesis of mammary and extramammary Paget's cells: an immunohistochemical study.Am J Dermatopathol 1989;11:318–22.

    Google Scholar 

  2. Berardi RS, Lee S, Chen HP. Perianal extramammary Paget's disease.Surg Gynecol Obstet 1988;167:359–66.

    CAS  PubMed  Google Scholar 

  3. Jensen SL, Sjolin KE, Shokouh-Amiri MH, Hagen K, Harling H. Paget's disease of the anal margin.Br J Surg 1988;75:1089–92.

    CAS  PubMed  Google Scholar 

  4. Schellenberger RR, Burford C, Kune GA. Perianal Paget's disease.Aust N Z J Surg 1988;58:727–32.

    CAS  PubMed  Google Scholar 

  5. Beck DE, Fazio VW. Perianal Paget's disease.Dis Colon Rectum 1987;30:263–6.

    CAS  PubMed  Google Scholar 

  6. Goldman S, Ihre T, Lagerstedt U, Svensson C. Perianal Paget's disease: report of five cases.Int J Colorectal Dis 1992;7:167–9.

    Article  CAS  PubMed  Google Scholar 

  7. Coldiron BM, Goldsmith BA, Robinson JK. Surgical treatment of extramammary Paget's disease. A report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision.Cancer 1991;67:93–8.

    Google Scholar 

  8. Sasaki M, Terada T, Nakunuma Y, Kono N, Kasahara Y, Watanabe K. Anorectal mucinous adenocarcinoma associated with latent perianal Paget's disease.Am J Gastroenterol 1990;85:199–202.

    CAS  PubMed  Google Scholar 

  9. Herzog U, von Flüe M, de Roche R, Curschellas E. Perianal extramammary Paget's disease. Report of two cases.Eur J Surg Oncol 1993; 19:569–73.

    Google Scholar 

  10. Petrelli NJ, Cebollera J, Rodriquez-Bigas M, Mang T. Photodynamic therapy in the management of neoplasms of the perianal skin.Arch Surg 1993;127:1536–8.

    Google Scholar 

  11. 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;24:73–8.

    CAS  PubMed  Google Scholar 

  12. Burrows NP, Jones DH, Hudson PM, Pye RJ. Treatment of extramammary Paget's disease by radiotherapy.Br J Dermatol 1995;132:970–2.

    CAS  PubMed  Google Scholar 

  13. Haberman HF, Goodall J, Llewellyn M. Extramammary Paget's disease.Can Med Assoc J 1978;118:161–2.

    CAS  PubMed  Google Scholar 

  14. Secco GB, Lapertosa G, Sertoli MR, Scarpati D. Riboli EB. Perineal Paget's disease: case report of an elderly patient treated with polychemotherapy and radiotherapy.Tumori 1984;70:381–3.

    CAS  PubMed  Google Scholar 

  15. Thirlby RC, Hammer CJ Jr, Galagan KS, Travaglini JJ, Picozzi VJ Jr. Perianal Paget's disease: successful treatment with combined chemoradiotherapy. Report of a case.Dis Colon Rectum 1990;33:150–2.

    Article  CAS  PubMed  Google Scholar 

  16. Shutze WP, Gleysteen JJ. Perianal Paget's disease. Classification and review of management: report of two cases.Dis Colon Rectum 1990;33:502–7.

    Article  CAS  PubMed  Google Scholar 

  17. Armitage MC, Jass JR, Richman PI, Thomson JP, Phillips RK. Paget's disease of the anus: a clinicopathological study.Br J Surg 1989; 76:60–3.

    CAS  PubMed  Google Scholar 

  18. Nishi M, Yoshida H, Setoyama M, Tashiro M. Immunohistochemical study of c-erbB-2 oncoprotein expression in extramammary Paget's disease.Dermatology 1994;188:100–2.

    CAS  PubMed  Google Scholar 

  19. Hollstein M, Sidransky D, Volgelstein B, Harris CC. p53 mutations in human cancers.Science 1991;253:49–53.

    CAS  PubMed  Google Scholar 

  20. Levine A, Momand J, Finlay C. The p53 tumour suppressor gene.Nature 1991;351:453–6.

    Article  CAS  PubMed  Google Scholar 

  21. Baker SJ, Preisinger AC, Jessup JM, et al. p53 gene mutations occur in combination with 17p allelic deletions as late events in colorectal tumorigenesis.Cancer Res 1990;50:7717–22.

    CAS  PubMed  Google Scholar 

  22. Finlay C, Hinds P, Levin A. The p53 procto-oncogen can act as a suppressor of transformation.Cell 1989;57:1083–93.

    Article  CAS  PubMed  Google Scholar 

  23. Reich NC, Levine AJ. Growth regulation of a cellular tumor antigen, p53, in nontransformed cells.Nature 1984;308:199–201.

    Article  CAS  PubMed  Google Scholar 

  24. Bruner J, Connelly J, Saya H. p53 protein immunostaining in routinely processed paraffin-embedded tissues.Mod Pathol 1993;6:189–94.

    CAS  PubMed  Google Scholar 

  25. Cattoretti G, Rilke F, Andreola S, D'Amato L, Delia D. p53 expression in breast cancer.Int J Cancer 1988;41:178–83.

    CAS  PubMed  Google Scholar 

  26. Yamaguchi A, Kurosaka Y, Fushida S, Kanno M, Ypnemura Y, Miwa K, Miyazaki I. Expression of p53 protein in colorectal cancer and its relationship to short-term prognosis.Cancer 1992;70:2778–84.

    CAS  PubMed  Google Scholar 

  27. Strauss RJ, Fazio. Bowen's disease of the anal and perianal area. A report and analysis of twelve cases.Am J Surg 1979;137:231–4.

    Article  CAS  PubMed  Google Scholar 

  28. More O, Hachisuka H, Maeyama Y, Sasai Y. A flow cytometric analysis of the DNA content of Paget cells in the epidermis and the tumor of extramammary Paget's disease.Acta Histochem 1995;97:99–104.

    Google Scholar 

  29. Miller LP, McCunniff AJ, Randall ME. An immunohistochemical study of perianal Paget's disease.Cancer 1992;69:2166–71.

    CAS  PubMed  Google Scholar 

  30. Kanitakis J, Thivolet J, Claudy A. p53 protein expression in mammary and extramammary Paget's disease.Anticancer Res 1993;13:2429–33.

    CAS  PubMed  Google Scholar 

  31. Nakamura G, Shikata N, Shoji T, Hatano T, Hioki K, Tsubura A. Immunohistochemical study of mammary and extramammary Paget's disease.Anticancer Res 1995;15:467–70.

    CAS  PubMed  Google Scholar 

  32. Wienecke R, Eckert F, Kaudewitz P, de Viragh PA, Heidl G, Volkenandt M. p53 protein in benign and malignant sweat gland tumors.Am J Dermatopathol 1994;16:126–9.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. From the Departments of Colorectal Surgery and Pathology, The Cleveland Clinic Foundation, Cleveland, Ohio

    Pierenrico Marchesa MD, Victor W. Fazio MD, Soledad Oliart MD, John R. Goldblum MD, Ian C. Lavery MD & Jeffrey W. Milsom MD

Authors
  1. Pierenrico Marchesa MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Victor W. Fazio MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Soledad Oliart MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. John R. Goldblum MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Ian C. Lavery MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Jeffrey W. Milsom MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Marchesa, P., Fazio, V.W., Oliart, S. et al. Long-term outcome of patients with perianal Paget's disease. Annals of Surgical Oncology 4, 475–480 (1997). https://doi.org/10.1007/BF02303671

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02303671

Key Words

Search

Navigation