Perianal Bowen\'s disease

Diseases of the

COLON & R E C T U M Vol. 31

June 1988

No. 6

Perianal Bowen's Disease DAVID E. BECK, MAJ, U S A F , MC, VICTOR W. FAZIO, M.B.B.S., F.A.C.S., F . R . A . C . S . , DAVID G. JAGELMAN, M.S.(LON), F.K.C.S.(ENG), IAN C. LAVERY, M.B.B.S., F.A.C.S., F.R.A.C.S.

Beck DE, Fazio VW, Jagelman DG, Laver~' IC. Perianal Bowen's disease. Dis Colon Rectum 1988;31:419-422. Thirty-three patients with perianal Bowen's disease were treated at the Cleveland Clinic Foundation from 1954 to 1986. Twenty-one patients were women and 12 were men, ranging in age from 30 to 69 years (mean, 48 years). Twenty patients (61 percent) presented with symptomatic perianal disease, while 13 patients (39 percent) were noted as having perianal Bowen's disease upon pathologic examination of routine hemorrhoidectomy specimens. Ten of the patients (30 percent) had prior histories of unrelated cancer. Twenty-seven patients were managed by wide local excision, three patients by simple excision, three patients by fulguration, and one patient by an abdominoperineal resection. D u r i n g a follow-up period averaging 3.7 years (range, 0.3 to 10 years), one patient developed a new invasive skin cancer while a second patient experienced a recurrence of perianal Bowen's disease. The characteristic gross appearance of this lesion and its failure to respond to conventional therapy should prompt the performance of a biopsy, which readily establishes the diagnosis. This experience confirms that wide local excision is adequate therapy for perianal Bowen's disease and that close clinical follow-up is necessary to identify disease recurrence or the development of a malignancy. [Key words: Bowen's disease, perianal; Anal cancer] BOWEN'S DISEASE IS a n u n c o m m o n i n t r a e p i t h e l i a l s q u a m o u s - c e l l c a r c i n o m a n a m e d after J o h n T. Bowen w h o , i n 1912, described two p a t i e n t s w i t h a t y p i c a l epithelial p r o l i f e r a t i o n of the skin. 1 T h e first case of p e r i a n a l B o w e n ' s disease was r e p o r t e d b y Vickers et al. in 19392 and, to date, there have been a p p r o x i m a t e l y 45 cases r e p o r t e d in the literature. T h i s i n s t i t u t i o n p r e v i o u s l y Poster presentation at the meeting of the American Society of Colon and Rectal Surgeons, Washington, D.C., April 5 to 10, 1987. The opinions expressed are those of the authors and do not reflect the opinions of the United States Air Force or the Department of Defense. Address reprint requests to Major Beck: SGHSG/Wilford Hall USAF Medical Center, Lackland AFB, Texas 78236.

From the Departments of Colorectal and General Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio, and Willord Hall US'IF Medical Center, Lackland AFB, Texas

r e p o r t e d 12 cases of p e r i a n a l B o w e n ' s disease. ~ T h e a u t h o r s n o w r e p o r t a n a d d i t i o n a l 21 p a t i e n t s a n d p r o v i d e further f o l l o w - u p o n the p r e v i o u s l y r e p o r t e d patients.

Materials a n d M e t h o d s A l l cases of p e r i a n a l B o w e n ' s disease treated at the C l e v e l a n d C l i n i c F o u n d a t i o n f r o m 1954 to 1986 were reviewed a n d p a t h o l o g i c s p e c i m e n s r e e x a m i n e d to conf i r m the diagnosis. C l i n i c a l f o l l o w - u p was o b t a i n e d b y p a t i e n t e x a m i n a t i o n s a n d p h o n e questionnaires. Results T h i r t y - t h r e e p a t i e n t s were i d e n t i f i e d as h a v i n g been treated for p e r i a n a l B o w e n ' s disease at the C l e v e l a n d C l i n i c F o u n d a t i o n f r o m 1954 to 1986. At the time of d i a g n o s i s , the p a t i e n t s r a n g e d in age f r o m 30 to 69 years (mean, 48 years). T w e n t y - o n e p a t i e n t s were w o m e n a n d 12 were men. T h i r t e e n p a t i e n t s (39 percent) were d i a g nosed w i t h p e r i a n a l B o w e n ' s disease after e x a m i n a t i o n of a h e m o r r h o i d e c t o m y s p e c i m e n , w h i l e the r e m a i n i n g 20 p a t i e n t s (61 percent) presented w i t h s y m p t o m a t i c peria n a l lesions t h a t h a d been p r e s e n t a n average of 34 m o n t h s (range, o n e m o n t h to ten years). Characteristic lesions i n s y m p t o m a t i c p a t i e n t s i n c l u d e d raised, irregular, scaly, b r o w n i s h - r e d p l a q u e s w i t h eczematoid features. T e n p a t i e n t s h a d histories of p r i o r cancer (seven w i t h

420

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BECK, ET AL.

TABLE1. #

Authors

Year

Pts

Vickers, et al3 Freund 4 Lutz5 Grodsky6 Grodsky7 Edwards8 Visaya et al. 9 Scoma and LevyL~ Strauss and Fazio3 Quan n Ramos et al. 12 Reynolds et al. 13 Greenall et al. TM Beck et al. TOTAL

1939 1941 1941 1954 1957 1965 1968 1975 1979 1980 1983 1984 1985 1987

3 1 1 3 1 1 1 2 12 12 7 6 7 33* 78

R e p o r t e d Series of Perianal B o w e n ' s Disease

Treatment

Mean Age

Sex

Exc

48 32 53 56 60 49 35 47 48 54 56 34

2-F M F 2-F M M M 2-F 6-F 7-F 5-F 6-F

1

48 48

June 1988

21-F 59%-F

1

Wle

Xrt

Assoc Cancer Oth

Prior

2

-

1

-

F/U (y)

-

1.9 0.7

-

-

1

-

-

1.4

-

-

0.1

4 -

-

2 1

2

7 2 11

2 10 14

0.5 3.2 4.8 3.4 1-5

2 7 12

5 6 3

F/U

1

1

10

1

2

1

3

Coexist

27 51

8

-

3

1 -

2 7 1 14

1 3

1.0

3.4

EXC: excision; XRT: radiotherapy; F/U: mean foll'ow-up; WLE: wide local excision; OTH: other; y: year. *Includes further follow-up of 12 patients reported in reference 3.

cervical cancer, two with l y m p h o m a , and one with a sarcoma). Twenty-seven patients were treated by wide local excision of the lesion, with 17 requiring split thickness skin grafts to cover the wounds. T h r e e patients u n d e r w e n t simple excision of' their lesions and three were treated by fulguration. O n e patient, w h o presented with a concomitant invasive anal carcinoma, was treated with an abdominoperineal resection. D u r i n g follow-up, w h i c h averaged 3.7 years (range, 0.3 to ten years), only one patient developed a new invasive skin cancer of the face. O n e of the patients treated by fulguration h a d a local recurrence of perianal Bowen's disease, requiring wide local excision of the recurrence.

Discussion

Patients with perianal Bowen's disease typically present with nonspecific complaints of anal itching, burning, or bleeding. T h e typical patient in this series was a w o m a n in her fifth decade of life with a significant probability of an antecedent unrelated cancer, findings in c o m m o n with previously reported series (Table 1). Examination of the p e r i n e u m in s y m p t o m a t i c patients usually reveals raised, irregular, scaly, brownish-red plaques with eczematoid features. T h e differential diagnosis of these lesions includes leukoplakia, Paget's disease, s q u a m o u s cell cancer, c o n d y l o m a a c u m i n a t u m , dermatitis, eczema, and d o w n w a r d spread of rectal carcinoma.15 Perianal Bowen's disease has a characteristic microscopic appearance of disordered epidermal hyperplasia with parakeratosis a n d hyperkeratosis in the superficial surface layers (Fig. 1). T h e m a l p i g h i a n cells also demon-

strate a disordered hyperplasia, with atypism and malign a n t dyskeratotic cells. Bowenoid cells (large atypical cells with haloed large h y p e r c h r o m a t i c nuclei) are present and mitotic figures are present in all layers.S, 7 In contrast to Paget's disease, the B o w e n o i d cells are PAS negative. A n y suspicious anal lesion, or one that fails to r e s p o n d to conventional therapy within a m o n t h , should be biopsied. A n adequate biopsy is essential to both c o n f i r m the diagnosis and to exclude an invasive carcinoma. A proper biopsy technique entails three or four full-thickness biopsies ( i . e . , i n c l u d i n g subcutaneous tissue) from the central p o r t i o n a n d edges of the lesion. T o assure that adequate biopsy depth is achieved, a sharp p u n c h biopsy and scalpel are used. Patients at the Cleveland Clinic F o u n d a t i o n were treated by several techniques. T h e early cases were m a n aged by simple excision, fulguration, a n d wide local excision. O n e of these patients experienced a local recurrence. T h e last 21 patients have been m a n a g e d by wide local excision, with n o recurrence of the Bowen's disease. T h u s , in the absence of invasive cancer, the authors prefer wide local excision. Adequate microscopically clear margins are i m p o r tant, as Bowen's cells m a y extend beyond the gross margins of the lesion. ~6 It has been the authors' practice to p e r f o r m biopsies 1 c m from the edge of the lesion a n d in all four q u a & a n t s of the p e r i n e u m (Fig. 2). As demonstrated in Fig. 2, 2 to 3 m m biopsies are taken at the dentate line, anal verge, and p e r i n e u m (approximately 2 to 3 c m from the anal verge). T h i s permits exact "lesion m a p p i n g , " a technique previously advocated by the authors.3,15 T h e wide local excision of the lesion is then completed. F o l l o w i n g removal of the specimen, the mar-

Volume 31 Number 6

PERIANAL BOWEN'S DISEASE

421

FIG.1. Microscopicappearance of Bowen's disease (hematoxylinand eosin; X 400).

gins of resection are examined by frozen section techniques to assure complete excision. T h e remaining wound defect can be handled in several ways. Small defects (involving less than 30 percent of the circumference of the anal canal) are closed primarily or left to heal by secondary intention. Defects greater than 50 percent circumferential are covered with a split thickness skin graft (either at the initial operation or 3 to 4 days later). For those defects 30 to 50 percent circumferential, the surgeon judges between these alternatives, depending on the elasticity of the tissues and evidence for degrees of anal stenosis (e.g., after previous hemorrhoidectomy). Since the original description of Bowen's disease, concern has frequently been voiced regarding the relationship of these epithelial lesions to invasive cancers, While early reports indicated that such a relationship existed,17 a recent analysis concluded that the published data did not support a relationship between Bowen's disease and the subsequent development of internal malignancies. 18 A survey of the collected experience with perianal Bowen's disease, also demonstrated that the incidence of subsequent malignancies was low. 19 T h e authors' experience

would seem to support this position, as only one patient developed a new malignancy during follow-up. A second concern has been the natural history of Bowen's disease. T h e clinical course of Bowen's disease has been relatively benign with progression toward invasive carcinoma in 2 to 6 percent of cases with long-term follow-up.19'20 Long-term follow-up is also thought to be necessary to prevent recurrence of perianal Bowen's disease. Limited experience with this disease, however, has hindered the development of a follow-up regimen. At the Cleveland Clinic Foundation, an annual complete physical examination, proctosigmoidoscopy, punch biopsy of any new lesion, and random biopsies at the edges of the splitthickness skin graft are performed. Colonoscopy is also performed at two to three yearly intervals. This experience confirms that wide local excision is adequate therapy for the treatment of perianal Bowen's disease in the absence of invasive carcinoma. An adequate initial evaluation, as well as long-term follow-up, are necessary to identify other potential malignancies in the patient with perianal Bowen's disease.

422

Dis. Col. & Rect.

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June 1988

References

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FIG. 2. Technique of quadrant biopsy of the perineum. 1: dentate line; 2: anal verge; 3: perineum.

Acknowledgment The authors thank Dr. Robert Petras for his assistance with the pathologic evaluation of these cases, Dr. Frank L. Weakley for allowing inclusion of his patients in this study, and Major Clyde E. McAuley for his assistance with preparation of the manuscript.

1. Bowen JT. Precancerous dermatoses: a study of two cases of chronic atypical epithelial proliferation. J Cutan Dis 1912;30: 241-55. 2. Vickers PM, Jackman RJ, McDonald JR. Anal carcinoma in situ: report of three cases. South Surgeon 1939;8:503-7. 3. Strauss RJ, Fazio VW. Bowen's disease of the anal and perianal area: a report and analysis of twelve cases. Am J Surg 1979;137: 231-4. 4. Freund MH. Bowen's disease of the anus and anal rection. Trans Am Proctol Soc 1941;42:149-52. 5. Lutz W. Ueber morbus Bowen vulvae et ani. Schweiz Med Wochenschr 1941;71:1298-1300. 6. Grodsky L. Bowen's disease of the anal region (squamous cell carcinoma in situ): report of three cases. Am J Surg 1954;88:7104. 7. Grodsky L. Intraepidermal cancer of the anus: evolution to invasive growth. Calif Med 1957;87:412-5. 8. Edwards M. Bowen's disease: a case report. Dis Colon Rectum 1965;8:297-9. 9. Visaya R Jr, Papadakis L, Calem WS. Bowen's disease in anoperineal region. NY State J Med 1968;68:306-7. 10. Scoma JA, Levy EI. Bowen's disease of the anus: report of two cases. Dis Colon Rectum 1975;18:137-40. 11. Quan SH. Uncommon malignant anal and rectal tumors. In: Stearns MW. Neoplasms of the colon, rectum, and anus. New York: John Wiley & Sons, 1980:121-2. 12. Ramos R, Salinas H, Tucker L. Conservative approach to the treatment of Bowen's disease of the anus. Dis Colon Rectum 1983;26:712-5. 13. Reynolds VH, Madden JJ, Franklin JD, Burnett LS, Jones HW III, Lynch JB. Preservation of anal function after total excision of the anal mucosa for Bowen's disease. Ann Surg 1984;199:563-8. 14. Greenall M J, Quan SH, Stearns MW, Urmacher C, DeCosse JJ. Epidermoid cancer of the anal margin: pathologic features, treatment, and clinical results. Am J Surg 1985;149:95-101. 15. Beck DE, Fazio VW. Perianal Paget's disease. Dis Colon Rectum 1987;30:263 -6. 16. Harrison EG Jr, Beahrs OH, Hill JR. Anal and perianal malignant neoplasms: pathology and treatment. Dis Colon Rectum 1966;9:255-67. 17. Graham JH, Helwig EB. Bowen's disease and its relationship to systemic cancer. Arch Dermatol 1961;83:738-58. 18. Arbesman H, Ransohoff DF. Is Bowen's disease a predictor for the development of internal malignancy? A methodological critique of the literature. JAMA 1987;257:516-8. 19. Morfing, TE, Abel ME, Gallagher DM. Perianal Bowen's disease and associated malignancies: results of a survey. Dis Colon Rectum 1987;30:782-5. 20. Stearns MW, Grodsky L, Harrison EG Jr, Quan S, Rob CG. Malignant anal lesions (panel discussion). Dis Colon Rectum 1966;9:315-27.