Skip to main content

Advertisement

SpringerLink
Log in

Perianaler Morbus Paget

Vorstellung von 6 Fällen und Auswertung der Literatur

Perianal Paget’s disease

Presentation of 6 cases and literature review

  • Standorte
  • Published:
coloproctology Aims and scope

Zusammenfassung

Fragestellung und Hintergrund

Der extramammäre Morbus Paget (EMPD) ist häufig mit Adnextumoren oder viszeralen synchronen oder metachronen malignen Tumoren assoziiert. Ziele dieser Arbeit waren die retrospektive Untersuchung der aus 6 EMPD-Fällen vorliegenden Ergebnisse sowie die Auswertung der Literatur.

Patienten und Methodik

Zwischen März 1996 und Dezember 2006 wurden 6 Patienten mit perianalem Morbus Paget in der Klinik der Autoren behandelt. In 3 Fällen war die Erkrankung auf die Epidermis beschränkt, in einem Fall lag eine Mikroinvasion der Dermis vor, während in einem weiteren Fall die Dermis infiltriert war. Letzterer war mit einem distalen rektalen Adenokarzinom assoziiert. Alle Patienten unterzogen sich einer weiten perianalen Exzision und einer Rekonstruktion mit einem Hauttransplantat. Die Autoren führten eine transanale Resektion des rektalen Adenokarzinoms durch.

Eine Auswertung der Literatur von 1990 bis 2008 ergab 193 Fälle von perianalem EMPD, wovon 112 intraepithelial/intradermal und 81 mit malignen Tumoren assoziiert waren. In 2 Fällen lagen bereits zuvor anorektale Adenokarzinome vor, in 48 Fällen waren sie synchron, und in 11 Fällen traten sie im Anschluss an die Diagnose auf.

Ergebnisse

In 3 Fällen traten Lokalrezidive auf, jedoch entwickelte kein Patient Metastasen. Fünf Patienten überlebten und sind erkrankungsfrei. Die Auswertung der Literatur erlaubt eine klare Identifikation des einfachen EMPD sowie der mit anorektalen Adenokarzinomen assoziierten Form, liefert jedoch nur wenige Informationen über die Fälle, die mit synchronen Adenokarzinomen der Adnexe assoziiert sind. Der Morbus Paget kann nach einer Radikaloperation erneut auftreten und hat ein gewisses Metastasierungspotenzial.

Schlussfolgerung

Bisher wurden noch keine klaren Leitlinien für die Diagnose des EMPD erstellt. Die Assoziation mit synchronen oder metachronen Karzinomen erfordert eine langfristige Nachbeobachtung mit häufigen klinischen, radiologischen und endoskopischen Kontrollen.

Abstract

Purpose

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 6 cases of EMPD and to review the literature.

Methods

Six patients with the perianal Paget’s disease had been treated in our division between March 1996 and December 2006. In 3 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 2 cases, synchronous in 48, and subsequent to diagnosis in 11.

Results

In 3 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.

Conclusion

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.

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

Abb. 1
Abb. 2

Literatur

  1. Shepherd V, Davidson EJ, Davies-Humphreys J (2005) Extramammary Paget’s disease. BJOG 112:273–279

    Article  PubMed  Google Scholar 

  2. Kanitakis J (2007) Mammary and extramammary Paget’s disease. J Eur Acad Dermatol Venereol 21:581–590

    Article  CAS  PubMed  Google Scholar 

  3. Demitsu T, Gonda K, Tanita M et al (1999) Extramammary Paget’s disease in two siblings. Br J Dermatol 141:951–952

    Article  CAS  PubMed  Google Scholar 

  4. Minicozzi A, Leopardi F, Segattini C et al (2008) Perianal Paget’s disease: five cases report. G Chir 29:469–474

    CAS  PubMed  Google Scholar 

  5. Amin R (1999) Perianal Paget’s disease. Br J Radiol 72:610–612

    CAS  PubMed  Google Scholar 

  6. Araki Y, Noake T, Hata H et al (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 46:1563–1565

    Article  PubMed  Google Scholar 

  7. Besa P, Rich TA, Delclos L et al (1992) Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys 24:73–78

    CAS  PubMed  Google Scholar 

  8. Delaunoit T, Neczyporenko F, Duttmann R et al (2004) Perianal Paget’s disease: case report and review of the literature. Acta Gastroenterol Belg 67:228–231

    PubMed  Google Scholar 

  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 60:1208–1213

    Article  PubMed  Google Scholar 

  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 22:170–179

    Article  CAS  PubMed  Google Scholar 

  11. Goldman S, Ihre T, Lagerstedt U, Svensson C (1992) Perianal Paget’s disease: report of five cases. Int J Colorectal Dis 7:167–169

    Article  CAS  PubMed  Google Scholar 

  12. Hassan I, Horgan AF, Nivatvongs S (2001) V-Y island flaps for repair of large perianal defects. Am J Surg 181:363–365

    Article  CAS  PubMed  Google Scholar 

  13. Hatta N, Morita R, Yamada M et al (2004) Sentinel lymph node biopsy in patients with extramammary Paget’s disease. Dermatol Surg 30:1329–1334

    Article  PubMed  Google Scholar 

  14. Helm KF, Goellner JR, Peters MS (1992) Immunohistochemical stains in extramammary Paget’s disease. Am J Dermatopathol 14:402–407

    Article  CAS  PubMed  Google Scholar 

  15. Hendi A, Brodland DG, Zitelli JA (2004) Extramammary Paget’s disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol 51:767–773

    Article  PubMed  Google Scholar 

  16. Honda Y, Egawa K (2005) Extramammary Paget’s disease not only mimicking but also accompanying condyloma acuminatum. A case report. Dermatology 210:315–318

    Article  PubMed  Google Scholar 

  17. Jabbar AS (2000) Perianal extramammary Paget’s disease. Eur J Surg Oncol 26:612–614

    Article  CAS  PubMed  Google Scholar 

  18. Khoubehi B, Schofield A, Leslie M et al (2001) Metastatic in-situ perianal Paget’s disease. J R Soc Med 94:137–138

    CAS  PubMed  Google Scholar 

  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 18:118–123

    Article  CAS  PubMed  Google Scholar 

  20. Lam DTY, Batista O, Weiss EG et al (2001) Staged and split-thickness skin gtaft for circumferential perianal Paget’s disease. Dis Colon Rectum 44:868–870

    Article  CAS  PubMed  Google Scholar 

  21. Marchesa P, Fazio VW, Oliart S et al (1997) Long-term outcome of patients with perianal Paget’s disease. Ann Surg Oncol 4:475–480

    Article  CAS  PubMed  Google Scholar 

  22. McCarter MD, Quan SH, Busam K et al (2003) Long-term outcome of perianal Paget’s disease. Dis Colon Rectum 46:612–616

    Article  PubMed  Google Scholar 

  23. Mehta NJ, Torno R, Sorra T (2000) Extramammary Paget’s disease. South Med J 93:713–715

    CAS  PubMed  Google Scholar 

  24. Mirer E, El Sayed F, Ammoury A et al (2006) Treatment of mammary and extramammary Paget’s skin disease with topical imiquimod. J Dermatolog Treat 17:167–171

    Article  PubMed  Google Scholar 

  25. Mochitomi Y, Sakamoto R, Gushi A et al (2005) Extramammary Paget’s disease/carcinoma successfully treated with a combination chemotherapy: report of two cases. J Dermatol 32:632–637

    PubMed  Google Scholar 

  26. Murakami K, Tanimura H, Ishimoto K et al (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 39:227–231

    Article  CAS  PubMed  Google Scholar 

  27. Nowak MA, Guerriere-Kovach P, Pathan A et al (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 122:1077–1081

    CAS  PubMed  Google Scholar 

  28. Panasiti V, Bottoni U, Devirgiliis V et al (2008) Intralesional interferon alfa- 2b as neoadjuvant treatment for perianal extramammary Paget’s disease. J Eur Acad Dermatol Venereol 22:522–523

    Article  CAS  PubMed  Google Scholar 

  29. Pierie JP, Choudry U, Muzikansky A et al (2003) Prognosis and management of extramammary Paget’s disease and the association with secondary malignancies. J Am Coll Surg 196:45–50

    Article  PubMed  Google Scholar 

  30. Rosin RD (1991) Paget’s disease of the anus. J R Soc Med 84:112–113

    CAS  PubMed  Google Scholar 

  31. Runfola MA, Weber TK, Rodriguez-Bigas MA et al (2000) Photodynamic therapy for residual neoplasms of the perianal skin. Dis Colon Rectum 43:499–502

    Article  CAS  PubMed  Google Scholar 

  32. Sarmiento JM, Wolff BG, Burgart LJ et al (1997) Paget’s disease of the perianal region – an aggressive disease? Dis Colon Rectum 40:1187–1194

    Article  CAS  PubMed  Google Scholar 

  33. Sasaki K, Nozaki M, Kikutchi Y et al (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 52:471–475

    Article  CAS  PubMed  Google Scholar 

  34. Shutze WP, Gleysteen JJ (1990) Perianal Paget’s disease. Classification and review of management: report of two cases. Dis Colon Rectum 33:502–507

    Article  CAS  PubMed  Google Scholar 

  35. St Peter SD, Pera M, Smith AA et al (2004) Wide local excision and split-thickness skin graft for circumferential Paget’s disease of the anus. Am J Surg 187:413–416

    Article  Google Scholar 

  36. Thirlby RC, Hammer CJ Jr, Galagan KA et al (1990) Perianal Paget’s disease: successful treatment with combined chemoradiotherapy. Report of a case. Dis Colon Rectum 33:150–152

    Article  CAS  PubMed  Google Scholar 

  37. Tulchinsky H, Zmora O, Brazowski E et al (2004) Extramammary Paget’s disease of the perianal region. Colorectal Dis 6:206–209

    Article  CAS  PubMed  Google Scholar 

  38. Vereecken P, Awada A, Ghanem G et al (2007) A therapeutic approach to perianal extramammary Paget’s disease: topical imiquimod can be useful to prevent or defer surgery. Med Sci Monit 13:CS75–CS77

    PubMed  Google Scholar 

  39. Yoon SN, Park IJ, Kim HC et al (2008) Extramammary Paget’s disease in Korea: its association with gastrointestinal neoplasms. Int J Colorectal Dis 23:1125–1130

    Article  PubMed  Google Scholar 

  40. Zibly Z, Haik J, Khaikin M et al (2006) Perianal Paget’s disease. J Cutan Med Surg 10:197–200

    PubMed  Google Scholar 

  41. Zollo JD, Zeitouni NC (2000) The Roswell Park Cancer Institute experience with extramammary Paget’s disease. Br J Dermatol 142:59–65

    Article  CAS  PubMed  Google Scholar 

  42. Bouzourene H, Saraga E (1997) Perianal Paget’s disease associated with anal canal adenocarcinoma and rectal villous adenoma. Histopathology 31:384–385

    Article  CAS  PubMed  Google Scholar 

  43. Brown RS, Lankester KJ, McCormack M et al (2002) Radiotherapy for perianal Paget’s disease. Clin Oncol 14:272–284

    Article  CAS  Google Scholar 

  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 20:199–200

    Article  Google Scholar 

  45. Guo L, Kuroda N, Miyazaki E et al (2004) Anal canal neuroendocrine carcinoma with Pagetoid extension. Pathol Int 54:630–635

    Article  PubMed  Google Scholar 

  46. Haga R, Suzuki H (2003) Rectal carcinoma associated with pagetoid phenomenon. Eur J Dermatol 13:93–94

    PubMed  Google Scholar 

  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 19:469–473

    CAS  PubMed  Google Scholar 

  48. Ikoma A, Kubo H, Ohno T et al (1996) Perianal extramammary Paget’s disease associated with primary linitis plastica of the rectum: report of a case. Surg Today 26:711–714

    Article  CAS  PubMed  Google Scholar 

  49. Koashi Y, Kitajima S, Schwartz RA, Tsuji T (1997) Perianal Paget’s disease years after rectal adenocarcinoma removal. Dermatol Surg 23:1032–1034

    Article  CAS  PubMed  Google Scholar 

  50. Kuan SF, Montag AG, Hart J et al (2001) Differential expression of mucin genes in mammary and extramammary Paget’s disease. Am J Surg Pathol 25:1469–1477

    Article  CAS  PubMed  Google Scholar 

  51. Kubota K, Akasu T, Nakanishi Y et al (1998) Perianal Paget’s disease associated with rectal carcinoma: a case report. Jpn J Clin Oncol 28:347–350

    Article  CAS  PubMed  Google Scholar 

  52. Lertprasertsuke N, Tsutsumi Y (1991) Latent perianal Paget’s disease associated with mucin-producing rectal adenocarcinoma. Report of two cases. Acta Pathol Jpn 41:386–393

    CAS  PubMed  Google Scholar 

  53. Makinen JM, Makinen MJ, Karttunen TJ (2002) Serrated adenocarcinoma of the rectum associated with perianal Paget’s disease: a case report. Histopathology 41:177–179

    Article  CAS  PubMed  Google Scholar 

  54. Miller LR, McCunniff AJ, Randall ME (1992) An immunohistochemical study of perianal Paget’s disease. Possible origins and clinical implications. Cancer 69:2166–2171

    Article  CAS  PubMed  Google Scholar 

  55. Sasaki M, Terada T, Nakanuma Y et al (1990) Anorectal mucinous adenocarcinoma associated with latent perianal Paget’s disease. Am J Gastroenterol 85:199–202

    CAS  PubMed  Google Scholar 

  56. Suenaga M, Oya M, Ueno M et al (2006) Anal canal carcinoma with Pagetoid spread: report of a case. Surg Today 36:666–669

    Article  PubMed  Google Scholar 

  57. Umemoto S, Inoue S, Amemiya T et al (1993) A case of perianal Paget’s disease associated with a sigmoid colon carcinoma. Gastroenterol Jpn 28:719–724

    CAS  PubMed  Google Scholar 

  58. Wong AY, Rahilly MA, Adams W, Lee CS (1998) Mucinous anal gland carcinoma with perianal pagetoid spread. Pathology 30:1–3

    Article  CAS  PubMed  Google Scholar 

  59. Yamamoto AI, Sato K, Wada T et al (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 29:185–189

    Article  Google Scholar 

  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:W26–W31

    Article  Google Scholar 

  61. Hernandez JM, Copeland EM (2007) Infiltrating apocrine adenocarcinoma with extramammary pagetoid spread. Am Surg 73:307–309

    PubMed  Google Scholar 

  62. Tsutsumida A, Yamamoto Y, Minakawa H et al (2003) Indications for lymph node dissection in the treatment of extramammary Paget’s disease. Dermatol Surg 29:21–24

    Article  PubMed  Google Scholar 

  63. Crowson AN, Magro CM, Mihm MC (2006) Malignant adnexal neoplasms. Mod Patology 19:S93–S126

    Article  Google Scholar 

  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 67:933–938

    Article  CAS  PubMed  Google Scholar 

  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 15:168–170

    CAS  PubMed  Google Scholar 

  66. O’Connor WJ, Lim KK, Zalla MJ et al (2003) Comparison of Mohs micrographic surgery and wide excision for extramammary Paget’s disease. Dermatol Surg 29:723–727

    Article  Google Scholar 

  67. Fujisawa Y, Umebayashi Y, Otsuka F (2006) Metastatic extramammary Paget’s disease successfully controlled with tumour dormancy therapy using docetaxel. Br J Dermatol 154:375–376

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. II Scuola di Specializzazione in Chirurgia Generale, I Divisione Clinicizzata di Chirurgia Generale, Ospedale Civile Maggiore, Università degli Studi di Verona, P.le A. Stefani, 1, 37126, Verona, Italien

    A. Minicozzi, G. Borzellino, R. Momo, F. Steccanella, F. Pitoni & G. de Manzoni

Authors
  1. A. Minicozzi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. G. Borzellino
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. R. Momo
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. F. Steccanella
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. F. Pitoni
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. G. de Manzoni
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to A. Minicozzi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Minicozzi, A., Borzellino, G., Momo, R. et al. Perianaler Morbus Paget. coloproctology 32, 209–215 (2010). https://doi.org/10.1007/s00053-010-0113-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00053-010-0113-8

Schlüsselwörter

Keywords

Search

Navigation