Perianal Paget’s disease: presentation of six cases and literature review

  • Annamaria Minicozzi
  • Giuseppe Borzellino
  • Rostand Momo
  • Francesca Steccanella
  • Federica Pitoni
  • Giovanni de Manzoni



Extramammary Paget’s disease (EMPD) is frequently associated with adnexal or visceral synchronous or metachronous malignancies. Our purpose was to evaluate, retrospectively, the results obtained in six cases of EMPD and to review the literature.


Six patients with the perianal Paget’s disease had been treated in our division between March 1996 and December 2006. In three cases, the disease was confined in the epidermis; in one case, there was a microinvasion of the dermis, while in another one the dermis was infiltrated. The last case was associated to a low rectal adenocarcinoma. All patients underwent wide perianal excision and reconstruction with skin graft. We performed a transanal resection of the rectal adenocarcinoma. A review of the literature from 1990 to 2008 revealed 193 cases of perianal EMPD, 112 were intraepithelial/intradermal while 81 were associated with malignancies. Anorectal adenocarcinoma was already existing in two cases, synchronous in 48, and subsequent to diagnosis in 11.


In three cases, the disease recurred locally, but no patient developed metastatic spread. Five patients survived and are free of disease. The review of the literature allows a clear identification of the primitive EMPD and the form associated to anorectal adenocarcinoma and little information about cases associated with synchronous adnexal adenocarcinoma. The Paget’s disease can relapse after radical surgery and has a capacity of metastatic spread.


Up to now, no clear guidelines have been established for the diagnosis of EMPD. The association with synchronous or metachronous carcinomas imposes a long-term follow-up with frequent clinical, radiological, and endoscopical controls.


Extramammary Paget’s disease Perianal Paget’s disease Perianal gland neoplasm Pagetoid phenomenon 


  1. 1.
    Shepherd V, Davidson EJ, Davies-Humphreys J (2005) Extramammary Paget’s disease. BJOG 112(3):273–279CrossRefPubMedGoogle Scholar
  2. 2.
    Kanitakis J (2007) Mammary and extramammary Paget’s disease. J Eur Acad Dermatol Venereol 21(5):581–590. ReviewPubMedGoogle Scholar
  3. 3.
    Demitsu T, Gonda K, Tanita M, Takahira K, Inoue T, Okada O, Yoneda K, Manabe M (1999) Extramammary Paget’s disease in two siblings. Br J Dermatol 141:951–952CrossRefPubMedGoogle Scholar
  4. 4.
    Minicozzi A, Leopardi F, Segattini C, Pitoni F, Steccanella F, De Manzoni G, Iannucci A, Governa M (2008) Perianal Paget’s disease: five cases report. G Chir 29(11–12):469–474PubMedGoogle Scholar
  5. 5.
    Amin R (1999) Perianal Paget’s disease. Br J Radiol 72(858):610–612PubMedGoogle Scholar
  6. 6.
    Araki Y, Noake T, Hata H, Momosaki K, Shirouzu K (2003) Perianal Paget’s disease treated with a wide excision and gluteal fold flap reconstruction guided by photodynamic diagnosis: report of a case. Dis Colon Rectum 46(11):1563–1565CrossRefPubMedGoogle Scholar
  7. 7.
    Besa P, Rich TA, Delclos L, Edwards CL, Ota DM, Wharton JT (1992) Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys 24(1):73–78PubMedGoogle Scholar
  8. 8.
    Delaunoit T, Neczyporenko F, Duttmann R, Deprez C, da Costa PM, de Koster E (2004) Perianal Paget’s disease: case report and review of the literature. Acta Gastroenterol Belg 67(2):228–231 ReviewPubMedGoogle Scholar
  9. 9.
    Fujii M, Terashi H, Hashikawa K, Tahara S (2007) Functional reconstruction of large anogenital defects with bilateral V-Y advancement posterior thigh fasciocutaneous flaps. J Plast Reconstr Aesthet Surg 60(11):1208–1213CrossRefPubMedGoogle Scholar
  10. 10.
    Goldblum JR, Hart WR (1998) Perianal Paget’s disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma. Am J Surg Pathol 22(2):170–179CrossRefPubMedGoogle Scholar
  11. 11.
    Goldman S, Ihre T, Lagerstedt U, Svensson C (1992) Perianal Paget’s disease: report of five cases. Int J Colorectal Dis 7(3):167–169CrossRefPubMedGoogle Scholar
  12. 12.
    Hassan I, Horgan AF, Nivatvongs S (2001) V-Y island flaps for repair of large perianal defects. Am J Surg 181(4):363–365CrossRefPubMedGoogle Scholar
  13. 13.
    Hatta N, Morita R, Yamada M, Echigo T, Hirano T, Takehara K, Ichiyanagi K, Yokoyama K (2004) Sentinel lymph node biopsy in patients with extramammary Paget’s disease. Dermatol Surg 30(10):1329–1334CrossRefPubMedGoogle Scholar
  14. 14.
    Helm KF, Goellner JR (1992) Peters MS Immunohistochemical stains in extramammary Paget’s disease. Am J Dermatopathol 14(5):402–407PubMedCrossRefGoogle Scholar
  15. 15.
    Hendi A, Brodland DG, Zitelli JA (2004) Extramammary Paget’s disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol 51(5):767–773CrossRefPubMedGoogle Scholar
  16. 16.
    Honda Y, Egawa K (2005) Extramammary Paget’s disease not only mimicking but also accompanying condyloma acuminatum. A case report. Dermatology 210(4):315–318CrossRefPubMedGoogle Scholar
  17. 17.
    Jabbar AS (2000) Perianal extramammary Paget’s disease. Eur J Surg Oncol 26(6):612–614CrossRefPubMedGoogle Scholar
  18. 18.
    Khoubehi B, Schofield A, Leslie M, Slevin ML, Talbot IC, Northover JM (2001) Metastatic in-situ perianal Paget’s disease. J R Soc Med 94(3):137–138PubMedGoogle Scholar
  19. 19.
    Kohler S, Smoller BR (1996) Gross cystic disease fluid protein-15 reactivity in extramammary Paget’s disease with and without associated internal malignancy. Am J Dermatopathol 18:118–123CrossRefPubMedGoogle Scholar
  20. 20.
    Lam DTY, Batista O, Weiss EG, Nogueras JJ, Wexner SD (2001) Staged and split-thickness skin gtaft for circumferential perianal Paget’s disease. Dis Colon Rectum 44:868–870CrossRefPubMedGoogle Scholar
  21. 21.
    Marchesa P, Fazio VW, Oliart S, Goldblum JR, Lavery IC, Milsom JW (1997) Long-term outcome of patients with perianal Paget’s disease. Ann Surg Oncol 4(6):475–480CrossRefPubMedGoogle Scholar
  22. 22.
    McCarter MD, Quan SH, Busam K, Paty PP, Wong D, Guillem JG (2003) Long-term outcome of perianal Paget’s disease. Dis Colon Rectum 46(5):612–616CrossRefPubMedGoogle Scholar
  23. 23.
    Mehta NJ, Torno R, Sorra T (2000) Extramammary Paget’s disease. South Med J 93(7):713–715PubMedGoogle Scholar
  24. 24.
    Mirer E, El Sayed F, Ammoury A, Lamant L, Messer L, Bazex J (2006) Treatment of mammary and extramammary Paget’s skin disease with topical imiquimod. J Dermatolog Treat 17(3):167–171CrossRefPubMedGoogle Scholar
  25. 25.
    Mochitomi Y, Sakamoto R, Gushi A, Hashiguchi T, Mera K, Matsushita S, Nishi M, Kanzaki T, Kanekura T (2005) Extramammary Paget’s disease/carcinoma successfully treated with a combination chemotherapy: report of two cases. J Dermatol 32(8):632–637PubMedGoogle Scholar
  26. 26.
    Murakami K, Tanimura H, Ishimoto K, Yamaue H, Yamade N, Shimamoto T (1996) Reconstruction with bilateral gluteus maximus myocutaneous rotation flap after wide local excision for perianal extramammary Paget’s disease. Report of two cases. Dis Colon Rectum 39(2):227–231CrossRefPubMedGoogle Scholar
  27. 27.
    Nowak MA, Guerriere-Kovach P, Pathan A, Campbell TE, Deppisch LM (1998) 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 122(12):1077–1081. ReviewPubMedGoogle Scholar
  28. 28.
    Panasiti V, Bottoni U, Devirgiliis V, Mancini M, Rossi M, Curzio M, Calvieri S (2008) Intralesional interferon alfa- 2b as neoadjuvant treatment for perianal extramammary Paget’s disease. JEADV 22:522–523PubMedGoogle Scholar
  29. 29.
    Pierie JP, Choudry U, Muzikansky A, Finkelstein DM, Ott MJ (2003) Prognosis and management of extramammary Paget’s disease and the association with secondary malignancies. J Am Coll Surg 196(1):45–50CrossRefPubMedGoogle Scholar
  30. 30.
    Rosin RD (1991) Paget’s disease of the anus. J R Soc Med 84(2):112–113PubMedGoogle Scholar
  31. 31.
    Runfola MA, Weber TK, Rodriguez-Bigas MA, Dougherty TJ, Petrelli NJ (2000) Photodynamic therapy for residual neoplasms of the perianal skin. Dis Colon Rectum 43(4):499–502CrossRefPubMedGoogle Scholar
  32. 32.
    Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM (1997) Paget’s disease of the perianal region–an aggressive disease? Dis Colon Rectum 40(10):1187–1194CrossRefPubMedGoogle Scholar
  33. 33.
    Sasaki K, Nozaki M, Kikutchi Y, Yamaki T, Soejima K (1999) Reconstruction of perianal skin defect using a V-Y advancement of bilateral gluteus maximus musculocutaneous flaps: reconstruction considering anal cleft and anal function. Br J Plast Surg 52(6):471–475CrossRefPubMedGoogle Scholar
  34. 34.
    Shutze WP, Gleysteen JJ (1990) Perianal Paget’s disease. Classification and review of management: report of two cases. Dis Colon Rectum 33(6):502–507. ReviewCrossRefPubMedGoogle Scholar
  35. 35.
    St Peter SD, Pera M, Smith AA, Leslie KO, Heppel J (2004) Wide local excision and split-Thickness skin graft for circumferential Paget’s disease of the anus. Am J Surg 187:413–416CrossRefPubMedGoogle Scholar
  36. 36.
    Thirlby RC, Hammer CJ Jr, Galagan KA, Travaglini JJ, Picozzi VJ Jr (1990) Perianal Paget’s disease: successful treatment with combined chemoradiotherapy. Report of a case. Dis Colon Rectum 33(2):150–152CrossRefPubMedGoogle Scholar
  37. 37.
    Tulchinsky H, Zmora O, Brazowski E, Goldman G, Rabau M (2004) Extramammary Paget’s disease of the perianal region. Colorectal Dis 6(3):206–209CrossRefPubMedGoogle Scholar
  38. 38.
    Vereecken P, Awada A, Ghanem G, da Costa CM, Larsimont D, Simoens C, da Costa PM, Hendlisz A (2007) A therapeutic approach to perianal extramammary Paget’s disease: topical imiquimod can be useful to prevent or defer surgery. Med Sci Mon 13(6):CS75–CS77Google Scholar
  39. 39.
    Yoon SN, Park IJ, Kim HC, Yu CS, Lee MW, Koh JK, Kim JW, Park YS, Kim JC (2008) Extramammary Paget’s disease in Korea: its association with gastrointestinal neoplasms. Int J Colorectal Dis 23(11):1125–1130CrossRefPubMedGoogle Scholar
  40. 40.
    Zibly Z, Haik J, Khaikin M, Barshack I, Zmora O (2006) Perianal Paget’s disease. J Cutan Med Surg 10(4):197–200PubMedGoogle Scholar
  41. 41.
    Zollo JD, Zeitouni NC (2000) The Roswell Park Cancer Institute experience with extramammary Paget’s disease. Br J Dermatol 142(1):59–65CrossRefPubMedGoogle Scholar
  42. 42.
    Bouzourene H, Saraga E (1997) Perianal Paget’s disease associated with anal canal adenocarcinoma and rectal villous adenoma. Histopathology 31(4):384–385CrossRefPubMedGoogle Scholar
  43. 43.
    Brown RS, Lankester KJ, McCormack M, Power DA, Spittle MF (2002) Radiotherapy for perianal Paget’s disease. Clin Oncol 14:272–284CrossRefGoogle Scholar
  44. 44.
    de la Portilla F, de la Rosa A, Bejarano D, Conde J (2005) Perianal Paget’s disease associated with rectal carcinoma: a rare report. Int J Colorectal Dis 20(2):199–200. Epub 2004 Sep 22CrossRefPubMedGoogle Scholar
  45. 45.
    Guo L, Kuroda N, Miyazaki E, Jin Y, Toi M, Hamauzu T, Hiroi M, Inoue T, Inoue A, Enzan H (2004) Anal canal neuroendocrine carcinoma with Pagetoid extension. Pathol Int 54(8):630–635CrossRefPubMedGoogle Scholar
  46. 46.
    Haga R, Suzuki H (2003) Rectal carcinoma associated with pagetoid phenomenon. Eur J Dermatol 13(1):93–94PubMedGoogle Scholar
  47. 47.
    Herzog U, von Flue M, de Roche R, Curschellas E (1993) Perianal extramammary Paget’s disease. Report of two cases. Eur J Surg Oncol 19(5):469–473PubMedGoogle Scholar
  48. 48.
    Ikoma A, Kubo H, Ohno T, Tanaka K, Yoshida A, Taira A (1996) Perianal extramammary Paget’s disease associated with primary linitis plastica of the rectum: report of a case. Surg Today 26(9):711–714CrossRefPubMedGoogle Scholar
  49. 49.
    Koashi Y, Kitajima S, Schwartz RA, Tsuji T (1997) Perianal Paget’s disease years after rectal adenocarcinoma removal. Dermatol Surg 23(11):1032–1034CrossRefPubMedGoogle Scholar
  50. 50.
    Kuan SF, Montag AG, Hart J, Krausz T, Recant W (2001) Differential expression of mucin genes in mammary and extramammary Paget’s disease. Am J Surg Pathol 25(12):1469–1477CrossRefPubMedGoogle Scholar
  51. 51.
    Kubota K, Akasu T, Nakanishi Y, Sugihara K, Fujita S, Moriya Y (1998) Perianal Paget’s disease associated with rectal carcinoma: a case report. Jpn J Clin Oncol 28(5):347–350. ReviewCrossRefPubMedGoogle Scholar
  52. 52.
    Lertprasertsuke N, Tsutsumi Y (1991) Latent perianal Paget’s disease associated with mucin-producing rectal adenocarcinoma. Report of two cases. Acta Pathol Jpn 41(5):386–393PubMedGoogle Scholar
  53. 53.
    Makinen JM, Makinen MJ, Karttunen TJ (2002) Serrated adenocarcinoma of the rectum associated with perianal Paget’s disease: a case report. Histopathology 41(2):177–179CrossRefPubMedGoogle Scholar
  54. 54.
    Miller LR, McCunniff AJ, Randall ME (1992) An immunohistochemical study of perianal Paget’s disease. Possible origins and clinical implications. Cancer 69(8):2166–2171CrossRefPubMedGoogle Scholar
  55. 55.
    Sasaki M, Terada T, Nakanuma Y, Kono N, Kasahara Y, Watanabe K (1990) Anorectal mucinous adenocarcinoma associated with latent perianal Paget’s disease. Am J Gastroenterol 85(2):199–202. ReviewPubMedGoogle Scholar
  56. 56.
    Suenaga M, Oya M, Ueno M, Yamamoto J, Yamaguchi T, Mizunuma N, Hatake K, Kato Y, Muto T (2006) Anal canal carcinoma with Pagetoid spread: report of a case. Surg Today 36(7):666–669CrossRefPubMedGoogle Scholar
  57. 57.
    Umemoto S, Inoue S, Amemiya T, Oka S, Hara T, Koizumi K, Nishida K (1993) A case of perianal Paget’s disease associated with a sigmoid colon carcinoma. Gastroenterol Jpn 28(5):719–724. ReviewPubMedGoogle Scholar
  58. 58.
    Wong AY, Rahilly MA, Adams W, Lee CS (1998) Mucinous anal gland carcinoma with perianal Pagetoid spread. Pathology 30(1):1–3CrossRefPubMedGoogle Scholar
  59. 59.
    Yamamoto AI, Sato K, Wada T, Takahashi H, Toyota N, Shibaki T, Yamazaki K, Tokusashi Y, Miyokawa N, Hajime I (2002) Fibroepithelioma-like changes occurring in perianal Paget’s disease with rectal mucinous carcinoma: case report and review of 49 cases of extramammary Paget’s disease. J Cutan Pathol 29:185–189CrossRefGoogle Scholar
  60. 60.
    Le Fur R, Mears L, Dannawi Z (2004) A peri-anal extramammary Paget’s disease associated with two well-differentiated invasive intramucosal sigmoid carcinomas, a very rare case: an immunohistochemical and clinical review of extramammary Paget’s disease. Ann R Coll Surg Engl 86(6):W26–W31PubMedGoogle Scholar
  61. 61.
    Hernandez JM, Copeland EM (2007) Infiltrating apocrine adenocarcinoma with extramammary pagetoid spread. Am Surg 73(3):307–309PubMedGoogle Scholar
  62. 62.
    Tsutsumida A, Yamamoto Y, Minakawa H, Yoshida T, Kokubu I, Sugihara T (2003) Indications for lymph node dissection in the treatment of extramammary Paget’s disease. Dermatol Surg 29(1):21–24CrossRefPubMedGoogle Scholar
  63. 63.
    Crowson AN, Magro CM, Mihm MC (2006) Malignant adnexal neoplasms. Modern Patology 19:S93–S126CrossRefGoogle Scholar
  64. 64.
    Coldiron BM, Goldsmith BA, Robinson JK (1991) 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 67(4):933–938. ReviewCrossRefPubMedGoogle Scholar
  65. 65.
    Murata Y, Kumano K (2005) Extramammary Paget’s disease of the genitalia with clinically clear margins can be adequately resected with 1 cm margin. Eur J Dermatol 15(3):168–170PubMedGoogle Scholar
  66. 66.
    O’Connor WJ, Lim KK, Zalla MJ, Gagnot M, Otley CC, Nguyen TH, Roenigk RK (2003) Comparison of mohs micrographic surgery and wide excision for extramammary Paget’s disease. Dermatol Surg 29(7):723–727CrossRefPubMedGoogle Scholar
  67. 67.
    Fujisawa Y, Umebayashi Y, Otsuka F (2006) Metastatic extramammary Paget’s disease successfully controlled with tumour dormancy therapy using docetaxel. Br J Dermatol 154(2):375–376CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Annamaria Minicozzi
    • 1
  • Giuseppe Borzellino
    • 1
  • Rostand Momo
    • 1
  • Francesca Steccanella
    • 1
  • Federica Pitoni
    • 1
  • Giovanni de Manzoni
    • 1
  1. 1.II Scuola di Specializzazione in Chirurgia Generale, I Divisione Clinicizzata di Chirurgia GeneraleOspedale Civile Maggiore, Università degli Studi di VeronaVeronaItaly

Personalised recommendations