ABSTRACT
Tumors of the anus and perianal skin are rare. Their presentation can vary and often mimics common benign anal pathology, thereby delaying diagnosis and appropriate and timely treatment. The anatomy of this region is complex because it represents the progressive transition from the digestive system to the skin with many different co-existing types of cells and tissues. Squamous cell carcinoma of the anal canal is the most frequent tumor found in the anal and perianal region. Less-frequent lesions include Bowen's and Paget's disease, basal cell carcinoma, melanoma, and adenocarcinoma. This article aims to review the clinical presentation, diagnostic evaluation, and treatment options for neoplasms of the anal canal and perianal skin.
KEYWORDS
Anal squamous-cell carcinoma - anal basal-cell carcinoma - anal melanoma - anal Paget's disease - anal Bowen's disease - anal adenocarcinoma
REFERENCES
-
1 Nivatvongs S. Perianal and anal canal neoplasms. In: Gordon P H, Nivatvongs S, eds. Colon, Rectum and Anus. 3rd ed. New York, NY: Informa Healthcare; 2007: 369-390
-
2 Welton M L, Varma M G. Anal cancer. In: Wolff B G, Fleshman J W, Beck D E, Pemberton J H, Wexner S D, eds. The ASCRS Textbook of Colon and Rectal Surgery. 1st ed. New York, NY: Springer; 2007: 482-500
-
3
Lengelé B, Scalliet P.
Anatomical bases for the radiological delineation of lymph node areas. Part III: pelvis and lower limbs.
Radiother Oncol.
2009;
92
(1)
22-33
-
4 Fenger D FM, Marti M C. Tumors of the anal canal. In: Hamilton S R, Aaltonen A L, ed. Pathology and Genetics of Tumors of the Digestive System. Lyon, France. IARC Press; 2000
-
5
Fenger C, Nielsen V T.
Intraepithelial neoplasia in the anal canal. The appearance and relation to genital neoplasia.
Acta Pathol Microbiol Immunol Scand [A].
1986;
94
(5)
343-349
-
6 Horner M, Ries L, Krapcho M et al.. SEER Cancer Statistics Review 1975–2006. Bethesda, MD: National Cancer Institute; 2007
-
7 Altekruse S F, Kosary C L, Krapcho M et al.. SEER Cancer Statistics Review 1975–2007. Available at: http://seer.cancer.gov/csr/1975_2007/ Accessed August 25, 2010
-
8 Edge S B, Byrd D R, Compton C C et al.. Anus. In: AJCC Cancer Staging Manual. New York, NY: American Joint Committee on Cancer and Springer; 2010: 165-173
-
9
Garrett K, Kalady M F.
Anal neoplasms.
Surg Clin North Am.
2010;
90
(1)
147-161
-
10
Franceschi S, De Vuyst H.
Human papillomavirus vaccines and anal carcinoma.
Curr Opin HIV AIDS.
2009;
4
(1)
57-63
-
11
Frisch M, Glimelius B, van den Brule A J et al..
Sexually transmitted infection as a cause of anal cancer.
N Engl J Med.
1997;
337
(19)
1350-1358
-
12
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
-
13
Marfing T E, Abel M E, Gallagher D M.
Perianal Bowen's disease and associated malignancies. Results of a survey.
Dis Colon Rectum.
1987;
30
(10)
782-785
-
14
Hoots B E, Palefsky J M, Pimenta J M, Smith J S.
Human papillomavirus type distribution in anal cancer and anal intraepithelial lesions.
Int J Cancer.
2009;
124
(10)
2375-2383
-
15
Palefsky J M, Holly E A, Ralston M L, Greenblatt R M, Greenblatt R M. Da Costa M .
Prevalence and risk factors for anal human papillomavirus infection in human immunodeficiency virus (HIV)-positive and high-risk HIV-negative women.
J Infect Dis.
2001;
183
(3)
383-391
-
16
Penn I.
Cancers of the anogenital region in renal transplant recipients. Analysis of 65 cases.
Cancer.
1986;
58
(3)
611-616
-
17
Daling J R, Sherman K J, Hislop T G et al..
Cigarette smoking and the risk of anogenital cancer.
Am J Epidemiol.
1992;
135
(2)
180-189
-
18
Frisch M.
On the etiology of anal squamous carcinoma.
Dan Med Bull.
2002;
49
(3)
194-209
-
19
Ryan D P, Compton C C, Mayer R J.
Carcinoma of the anal canal.
N Engl J Med.
2000;
342
(11)
792-800
-
20
Gerard J P, Chapet O, Samiei F et al..
Management of inguinal lymph node metastases in patients with carcinoma of the anal canal: experience in a series of 270 patients treated in Lyon and review of the literature.
Cancer.
2001;
92
(1)
77-84
-
21 Keightly M, Williams N. Malignant tumours of the anal canal and anus. In: Surgery of the Anus, Rectum and Colon. 3rd ed. Philadelphia, PA: Saunders; 2008: 567-590
-
22
Damin D C, Rosito M A, Schwartsmann G.
Sentinel lymph node in carcinoma of the anal canal: a review.
Eur J Surg Oncol.
2006;
32
(3)
247-252
-
23
Mistrangelo M, Pelosi E, Bellò M et al..
Comparison of positron emission tomography scanning and sentinel node biopsy in the detection of inguinal node metastases in patients with anal cancer.
Int J Radiat Oncol Biol Phys.
2010;
77
(1)
73-78
-
24
Clark M A, Hartley A, Geh J I.
Cancer of the anal canal.
Lancet Oncol.
2004;
5
(3)
149-157
-
25
Boman B M, Moertel C G, O'Connell M J et al..
Carcinoma of the anal canal. A clinical and pathologic study of 188 cases.
Cancer.
1984;
54
(1)
114-125
-
26
Nigro N D, Vaitkevicius V K, Considine Jr B.
Combined therapy for cancer of the anal canal: a preliminary report.
Dis Colon Rectum.
1974;
17
(3)
354-356
-
27
UKCCCR Anal Cancer Trial Working Party. UK Co-ordinating Committee on Cancer Research .
Epidermoid anal cancer: results from the UKCCCR randomised trial of radiotherapy alone versus radiotherapy, 5-fluorouracil, and mitomycin.
Lancet.
1996;
348
(9034)
1049-1054
-
28
Northover J, Glynne-Jones R, Sebag-Montefiore D et al..
Chemoradiation for the treatment of epidermoid anal cancer: 13-year follow-up of the first randomised UKCCCR Anal Cancer Trial (ACT I).
Br J Cancer.
2010;
102
(7)
1123-1128
-
29
Ajani J A, Winter K A, Gunderson L L et al..
Fluorouracil, mitomycin, and radiotherapy vs fluorouracil, cisplatin, and radiotherapy for carcinoma of the anal canal: a randomized controlled trial.
JAMA.
2008;
299
(16)
1914-1921
-
30
Bartelink H, Roelofsen F, Eschwege F et al..
Concomitant radiotherapy and chemotherapy is superior to radiotherapy alone in the treatment of locally advanced anal cancer: results of a phase III randomized trial of the European Organization for Research and Treatment of Cancer Radiotherapy and Gastrointestinal Cooperative Groups.
J Clin Oncol.
1997;
15
(5)
2040-2049
-
31
Flam M, John M, Pajak T F et al..
Role of mitomycin in combination with fluorouracil and radiotherapy, and of salvage chemoradiation in the definitive nonsurgical treatment of epidermoid carcinoma of the anal canal: results of a phase III randomized intergroup study.
J Clin Oncol.
1996;
14
(9)
2527-2539
-
32
Das P, Bhatia S, Eng C et al..
Predictors and patterns of recurrence after definitive chemoradiation for anal cancer.
Int J Radiat Oncol Biol Phys.
2007;
68
(3)
794-800
-
33
Czito B G, Willett C G.
Current management of anal canal cancer.
Curr Oncol Rep.
2009;
11
(3)
186-192
-
34
Fleshner P R, Chalasani S, Chang G J, Levien D H, Hyman N H, Buie W D. Standards Practice Task Force, American Society of Colon and Rectal Surgeons .
Practice parameters for anal squamous neoplasms.
Dis Colon Rectum.
2008;
51
(1)
2-9
-
35
Christensen A F, Nyhuus B, Nielsen M B.
Interobserver and intraobserver variation of two-dimensional and three-dimensional anal endosonography in the evaluation of recurrent anal cancer.
Dis Colon Rectum.
2009;
52
(3)
484-488
-
36
Akbari R P, Paty P B, Guillem J G et al..
Oncologic outcomes of salvage surgery for epidermoid carcinoma of the anus initially managed with combined modality therapy.
Dis Colon Rectum.
2004;
47
(7)
1136-1144
-
37
Ferenschild F T, Vermaas M, Hofer S O, Verhoef C, Eggermont A M, de Wilt J H.
Salvage abdominoperineal resection and perineal wound healing in local recurrent or persistent anal cancer.
World J Surg.
2005;
29
(11)
1452-1457
-
38
Ghouti L, Houvenaeghel G, Moutardier V et al..
Salvage abdominoperineal resection after failure of conservative treatment in anal epidermoid cancer.
Dis Colon Rectum.
2005;
48
(1)
16-22
-
39
Nilsson P J, Svensson C, Goldman S, Glimelius B.
Salvage abdominoperineal resection in anal epidermoid cancer.
Br J Surg.
2002;
89
(11)
1425-1429
-
40
Renehan A G, Saunders M P, Schofield P F, O'Dwyer S T.
Patterns of local disease failure and outcome after salvage surgery in patients with anal cancer.
Br J Surg.
2005;
92
(5)
605-614
-
41
Schiller D E, Cummings B J, Rai S et al..
Outcomes of salvage surgery for squamous cell carcinoma of the anal canal.
Ann Surg Oncol.
2007;
14
(10)
2780-2789
-
42
Sunesen K G, Buntzen S, Tei T, Lindegaard J C, Nørgaard M, Laurberg S.
Perineal healing and survival after anal cancer salvage surgery: 10-year experience with primary perineal reconstruction using the vertical rectus abdominis myocutaneous (VRAM) flap.
Ann Surg Oncol.
2009;
16
(1)
68-77
-
43
Stewart D, Yan Y, Kodner I J et al..
Salvage surgery after failed chemoradiation for anal canal cancer: should the paradigm be changed for high-risk tumors?.
J Gastrointest Surg.
2007;
11
(12)
1744-1751
-
44
Lefevre J H, Parc Y, Kernéis S et al..
Abdomino-perineal resection for anal cancer: impact of a vertical rectus abdominis myocutaneus flap on survival, recurrence, morbidity, and wound healing.
Ann Surg.
2009;
250
(5)
707-711
-
45
Colvin M, Delis A, Bracamonte E, Villar H, Leon Jr L R.
Infiltrating adenocarcinoma arising in a villous adenoma of the anal canal.
World J Gastroenterol.
2009;
15
(28)
3560-3564
-
46
Chang G J, Gonzalez R J, Skibber J M, Eng C, Das P, Rodriguez-Bigas M A.
A twenty-year experience with adenocarcinoma of the anal canal.
Dis Colon Rectum.
2009;
52
(8)
1375-1380
-
47
Kounalakis N, Artinyan A, Smith D, Mojica-Manoso P, Paz B, Lai L L.
Abdominal perineal resection improves survival for nonmetastatic adenocarcinoma of the anal canal.
Ann Surg Oncol.
2009;
16
(5)
1310-1315
-
48
Beal K P, Wong D, Guillem J G et al..
Primary adenocarcinoma of the anus treated with combined modality therapy.
Dis Colon Rectum.
2003;
46
(10)
1320-1324
-
49
Belkacémi Y, Berger C, Poortmans P Rare Cancer Network et al.
Management of primary anal canal adenocarcinoma: a large retrospective study from the Rare Cancer Network.
Int J Radiat Oncol Biol Phys.
2003;
56
(5)
1274-1283
-
50
Sahai A, Kodner I J.
Premalignant neoplasms and squamous cell carcinoma of the anal margin.
Clin Colon Rectal Surg.
2006;
19
(2)
88-93
-
51
Minicozzi A, Borzellino G, Momo R, Steccanella F, Pitoni F, de Manzoni G.
Perianal Paget's disease: presentation of six cases and literature review.
Int J Colorectal Dis.
2010;
25
(1)
1-7
-
52
Beck D E, Fazio V W.
Perianal Paget's disease.
Dis Colon Rectum.
1987;
30
(4)
263-266
-
53
McCarter M D, Quan S H, Busam K, Paty P P, Wong D, Guillem J G.
Long-term outcome of perianal Paget's disease.
Dis Colon Rectum.
2003;
46
(5)
612-616
-
54
Mirer E, El Sayed F, Ammoury A, Lamant L, Messer L, Bazex J.
Treatment of mammary and extramammary Paget's skin disease with topical imiquimod.
J Dermatolog Treat.
2006;
17
(3)
167-171
-
55
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
(10)
1187-1194
-
56
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
-
57
Sarmiento J M, Wolff B G, Burgart L J, Frizelle 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
-
58
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;
13
(5)
951-955
-
59
Runfola M A, Weber T K, Rodriguez-Bigas M A, Dougherty T J, Petrelli N J.
Photodynamic therapy for residual neoplasms of the perianal skin.
Dis Colon Rectum.
2000;
43
(4)
499-502
-
60
Vereecken P, Awada A, Ghanem G et al..
A therapeutic approach to perianal extramammary Paget's disease: topical imiquimod can be useful to prevent or defer surgery.
Med Sci Monit.
2007;
13
(6)
CS75-CS77
-
61
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
-
62
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
(10)
1655-1660
discussion 1660-1661
-
63
Cox N H, Eedy D J, Morton C A. Therapy Guidelines and Audit Subcommittee, British Association of Dermatologists .
Guidelines for management of Bowen's disease: 2006 update.
Br J Dermatol.
2007;
156
(1)
11-21
-
64
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
-
65 Edge S B, Byrd D R, Compton C C et al.. Cutaneous squamous cell carcinoma and other cutaneous carcinomas. In: AJCC Cancer Staging Manual. New York, NY: American Joint Committee on Cancer and Springer; 2010: 301-314
-
66
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
-
67
Fuchshuber P R, Rodriguez-Bigas M, Weber T, Petrelli N J.
Anal canal and perianal epidermoid cancers.
J Am Coll Surg.
1997;
185
(5)
494-505
-
68
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
-
69
Bieri S, Allal A S, Kurtz J M.
Sphincter-conserving treatment of carcinomas of the anal margin.
Acta Oncol.
2001;
40
(1)
29-33
-
70
Gibson G E, Ahmed I.
Perianal and genital basal cell carcinoma: a clinicopathologic review of 51 cases.
J Am Acad Dermatol.
2001;
45
(1)
68-71
-
71
Nielsen O V, Jensen S L.
Basal cell carcinoma of the anus-a clinical study of 34 cases.
Br J Surg.
1981;
68
(12)
856-857
-
72
Paterson C A, Young-Fadok T M, Dozois R R.
Basal cell carcinoma of the perianal region: 20-year experience.
Dis Colon Rectum.
1999;
42
(9)
1200-1202
-
73
Iddings D M, Fleisig A J, Chen S L, Faries M B, Morton D L.
Practice patterns and outcomes for anorectal melanoma in the USA, reviewing three decades of treatment: is more extensive surgical resection beneficial in all patients?.
Ann Surg Oncol.
2010;
17
(1)
40-44
-
74
Kiran R P, Rottoli M, Pokala N, Fazio V W.
Long-term outcomes after local excision and radical surgery for anal melanoma: data from a population database.
Dis Colon Rectum.
2010;
53
(4)
402-408
-
75
Bullard K M, Tuttle T M, Rothenberger D A et al..
Surgical therapy for anorectal melanoma.
J Am Coll Surg.
2003;
196
(2)
206-211
-
76
Thibault C, Sagar P, Nivatvongs S, Ilstrup D M, Wolff B G.
Anorectal melanoma—an incurable disease?.
Dis Colon Rectum.
1997;
40
(6)
661-668
-
77
Weyandt G H, Eggert A O, Houf M, Raulf F, Bröcker E B, Becker J C.
Anorectal melanoma: surgical management guidelines according to tumour thickness.
Br J Cancer.
2003;
89
(11)
2019-2022
-
78
Yeh J J, Shia J, Hwu W J et al..
The role of abdominoperineal resection as surgical therapy for anorectal melanoma.
Ann Surg.
2006;
244
(6)
1012-1017
Eric J DozoisM.D.
Division of Colon and Rectal Surgery, Mayo Clinic, Gonda 9 South
200 First St. SW, Rochester, MN 55905
eMail: Dozois.eric@mayo.edu
