Clin Colon Rectal Surg 2009; 22(2): 127-135
DOI: 10.1055/s-0029-1223845
© Thieme Medical Publishers

Malignancies of the Anal Margin and Perianal Skin

E. Dawn Wietfeldt1 , James Thiele1 , 2
  • 1Department of Surgery, Section of Colorectal Surgery, Southern Illinois University School of Medicine, Springfield, Illinois
  • 2Department of Colon and Rectal Surgery, Springfield Clinic, Springfield, Illinois
Further Information

Publication History

Publication Date:
27 May 2009 (online)

ABSTRACT

Malignancies of the anal margin and perianal skin are relatively uncommon lesions, comprising 3 to 4% of all anorectal malignancies. Commonly included in this group of cancers are Bowen's disease (intraepithelial squamous cell cancer), perianal Paget's disease (intraepithelial adenocarcinoma), invasive squamous cell cancer, basal cell cancer, and malignant melanoma. Buschke-Lowenstein tumor, or giant condyloma acuminatum, is not always included because this lesion is technically benign, although it displays aggressive local invasive behavior that makes it difficult to manage.

Complaints are usually nonspecific, such as itching or burning, bleeding, pain, drainage, or a mass. Proper diagnosis requires a high index of suspicion on the part of the surgeon. Innocent local irritations will resolve in a short time with appropriate therapy; those that persist must be biopsied for tissue diagnosis.

Wide local excision is the mainstay of treatment for early stage tumors as it preserves continence and obtains adequate local control. Adjunct therapies have been utilized in more advanced or recurrent lesions, including radiotherapy, photodynamic therapy, and imiquimod. All have met with a fair amount of success in controlling local disease; however, the number of patients treated in each instance is small, making it difficult to design an evidence-based treatment strategy. Invasion and metastasis are relatively rare in this group of neoplasms; perianal Paget's disease has the highest risk of associated underlying neoplasm. The most important consideration in developing a treatment strategy is which strategy would achieve the best clinical result with the least morbidity to the patient.

REFERENCES

  • 1 Newlin H E, Zlotecki R A, Morris C G, Hochwald S N, Riggs C E, Mendenhall W M. Squamous cell carcinoma of the anal margin.  J Surg Oncol. 2004;  86(2) 55-62, discussion 63
  • 2 Mendenhall W M, Zlotecki R A, Vauthey J N, Copeland III E M. Squamous cell carcinoma of the anal margin.  Oncology (Williston Park). 1996;  10(12) 1843-1848, discussion 1848, 1853–1854
  • 3 Khanfir K, Ozsahin M, Bieri S, Cavuto C, Mirimanoff R O, Zouhair A. Patterns of failure and outcome in patients with carcinoma of the anal margin.  Ann Surg Oncol. 2008;  15(4) 1092-1098
  • 4 Chapet O, Gerard J P, Mornex F et al.. Prognostic factors of squamous cell carcinoma of the anal margin treated by radiotherapy: the Lyon experience.  Int J Colorectal Dis. 2007;  22(2) 191-199
  • 5 Quan S. Anal cancers squamous and melanoma.  Cancer. 1992;  70(suppl 5) 1384-1389
  • 6 Welton M, Varma M. Anal cancer. In: Wolff B, Fleshman J, Beck D, Pemberton J, Wexner S, et al The ASCRS Textbook of Colon and Rectal Surgery. New York; Springer Science + Business Media 2007: 482-500
  • 7 Chawla A K, Willett C G. Squamous cell carcinoma of the anal canal and anal margin.  Hematol Oncol Clin North Am. 2001;  15(2) 321-344, vi
  • 8 Gordon P H, Nivatvongs S. Principles and Practice of Surgery for the Colon, Rectum, and Anus. 2nd ed. St. Louis, MO; Quality Medical Publishing Inc. 1999: 450-451
  • 9 Grabenbauer G G, Kessler H, Matzel K E, Sauer R, Hohenberger W, Schneider I H. Tumor site predicts outcome after radiochemotherapy in squamous-cell carcinoma of the anal region: long-term results of 101 patients.  Dis Colon Rectum. 2005;  48(9) 1742-1751
  • 10 Manstein C, Gottlieb N, Manstein M, Manstein G. Giant basal cell carcinoma: a series of seven t3 tumors without metastasis.  Plastics and Reconstructive Surgery. 2000;  106(3) 653-656
  • 11 Moore H G, Guillem J G. Anal neoplasms.  Surg Clin North Am. 2002;  82(6) 1233-1251
  • 12 Wang S Q, Goldberg L H. Multiple polypoid basal cell carcinomas on the perineum of a patient with basal cell nevus syndrome.  J Am Acad Dermatol. 2007;  57 (2, Suppl) S36-S37
  • 13 Beck D E. Perianal Paget's disease and Bowen's disease of the anus.  Semin Colon Rectal Surgery. 1995;  6 143-149
  • 14 Tjandra J. Perianal Paget's disease. Report of three cases.  Dis Colon Rectum. 1988;  31(6) 462-466
  • 15 Beck D E, Fazio V W. Perianal Paget's disease.  Dis Colon Rectum. 1987;  30(4) 263-266
  • 16 Goldman S, Ihre T, Lagerstedt U, Svensson C. Perianal Paget's disease: report of five cases.  Int J Colorectal Dis. 1992;  7(3) 167-169
  • 17 Zollo J D, Zeitouni N C. The Roswell Park Cancer Institute experience with extramammary Paget's disease.  Br J Dermatol. 2000;  142(1) 59-65
  • 18 Marchesa P, Fazio V W, Oliart S, Goldblum J R, Lavery I C, Milsom J W. Long-term outcome of patients with perianal Paget's disease.  Ann Surg Oncol. 1997;  4(6) 475-480
  • 19 Chanda J J. Extramammary Paget's disease: prognosis and relationship to internal malignancy.  J Am Acad Dermatol. 1985;  13(6) 1009-1014
  • 20 Gaertner W B, Hagerman G F, Goldberg S A, Finne C O. Perianal Paget's disease treated with wide excision and gluteal skin flap reconstruction: report of a case and review of the literature.  Dis Colon Rectum. 2008;  51 1842-1845
  • 21 McCarter M D, Quan S H, Busam K, Paty P P, Wong D, Guilem J G. Long term outcome of perianal Paget's disease.  Dis Colon Rectum. 2003;  46 612-616
  • 22 St Peter S D, Pera M, Smith A A, Leslie K O, Heppell J. Wide local excision and split thickness skin graft for circumferential Paget's disease.  Am J Surg. 2004;  187(3) 413-416
  • 23 Shutze W P, Gleysteen J J. Perianal Paget's disease. Classification and review of management: report of two cases.  Dis Colon Rectum. 1990;  33(6) 502-507
  • 24 Shieh S, Dee A S, Cheney R T, Frawley N P, Zeitouni N C, Oseroff A R. Photodynamic therapy for the treatment of extramammary Paget's disease.  Br J Dermatol. 2002;  146 100-105
  • 25 Moreno-Arias G A, Conill C, Castells-Mas A, Arenas M, Grimalt R. Radiotherapy for genital extramammary Paget's disease in-situ.  Dermatol Surg. 2001;  27 587-590
  • 26 Burrows N P, Jones D H, Hudson P M, Pye R J. Treatment of extramammary Paget's disease by radiotherapy.  Br J Dermatol. 1995;  132 970-972
  • 27 Sarmiento J M, Wolff B G, Burgart L J, Frizelle F A, Ilstrup D M. Paget's disease of the perianal region – an aggressive disease?.  Dis Colon Rectum. 1997;  40 1187-1194
  • 28 Cleary R K, Schaldenbrand J D, Fowler J J, Schuler J M, Lampman R M. Perianal Bowen's disease and anal intraepithelial neoplasia: review of the literature.  Dis Colon Rectum. 1999;  42(7) 945-951
  • 29 Marchesa P, Fazio V W, Oliart S, Goldblum J R, Lavery I C. Perianal Bowen's disease: a clinicopathologic study of 47 patients.  Dis Colon Rectum. 1997;  40(11) 1286-1293
  • 30 Margenthaler J A, Dietz D W, Mutch M G, Birnbaum E H, Kodner I J, Fleshman J W. Outcomes, risk of other malignancies, and need for formal mapping procedures in patients with perianal Bowen's disease.  Dis Colon Rectum. 2004;  47 1655-1660 discussion 1660-1651
  • 31 Sarmiento J M, Wolff B G, Burgart L J, Frizelee F A, Ilstrup D M. Perianal Bowen's disease. Associated tumors, human papillomavirus, surgery and other controversies.  Dis Colon Rectum. 1997;  40(8) 912-918
  • 32 Cox N H, Eedy D J, Morton C A. Guidelines for management of Bowen's disease: 2006 update.  Br J Dermatol. 2007;  156 11-21
  • 33 Cleary R K, Schaldenbrand J D, Fowler J J, Schuler J M, Lampman R M. Treatment options for perianal Bowen's disease: Survey of American Society of Colon and Rectal Surgeons Members.  Am Surg. 2000;  66(7) 686-688
  • 34 Hassan I, Horgan A F, Nivatvongs S. V-Y island flaps for repair of large perianal defects.  Am J Surg. 2001;  181(4) 363-365
  • 35 Conklin A, Hassan I, Chua H K et al.. Long term functional and quality of life outcomes of patients after repair of large perianal skin defects for Paget's and Bowen's disease.  J Gastrointest Surg. 2009;  , Epub ahead of print
  • 36 Patel G K, Goodwin R, Chawla M et al.. Imiquimod 5% cream monotherapy for cutaneous squamous cell carcinoma in situ (Bowen's disease): a randomized, double-blind, placebo-controlled trial.  J Am Acad Dermatol. 2006;  54(6) 1025-1032
  • 37 Bargman H, Hochman J. Topical treatment of Bowen's disease with 5-fluorouracil.  J Cutan Med Surg. 2003;  7(2) 101-105
  • 38 Graham B D, Jetmore A B, Foote J E, Arnold L K. Topical 5-fluorouracil in the management of extensive anal Bowen's disease: a preferred approach.  Dis Colon Rectum. 2005;  48(3) 444-450
  • 39 Gupta A K, Browne M, Bluhm R. Imiquimod: a review.  J Cutan Med Surg. 2002;  6(6) 554-560
  • 40 Rosen T, Harting M, Gibson M. Treatment of Bowen's disease with topical 5% imiquimod cream: retrospective study.  Dermatol Surg. 2007;  33 427-431 discussion 431-422
  • 41 Peris K, Micantonio T, Fargnoli M C, Lozzi G P, Chimenti S. Imiquimod 5% cream in the treatment of Bowen's disease and invasive squamous cell carcinoma.  J Am Acad Dermatol. 2006;  55(2) 324-327
  • 42 De Toma G, Cavallaro G, Bitonti A, Polistena A, Onesti M G, Scuderi N. Surgical management of perianal giant condyloma acuminatum (Buschke-Löwenstein tumor). Report of three cases.  Eur Surg Res. 2006;  38(4) 418-422
  • 43 Chu Q D, Vezeridis M P, Libbey N P, Wanebo H J. Giant condyloma acuminatum (Buschke-Lowenstein tumor) of the anorectal and perianal regions. Analysis of 42 cases.  Dis Colon Rectum. 1994;  37(9) 950-957
  • 44 Cintron J R. Buschke-Lowenstein tumor of the perianal and anorectal region.  Semin Colon Rectal Surg. 1995;  6 135-139
  • 45 Tytherleigh M G, Birtle A J, Cohen C E, Glynne-Jones R, Livingstone J, Gilbert J. Combined surgery and chemoradiation as a treatment for the Buschke-Löwenstein tumour.  Surgeon. 2006;  4(6) 378-383

James ThieleM.D. 

Department of Colon and Rectal Surgery, Springfield Clinic

800 N. First St., Springfield, IL 62702

Email: thiele1969@gmail.com