Bowen’s Disease
Bowen’s disease, first described by John T. Bowen5 in 1912, is an intraepidermal squamous cell carcinoma (carcinoma in situ) that occurs most frequently on the face, hands, and trunk, with the perianal location being the least common.6 Perianal Bowen’s disease is considered a rare, slow-growing, premalignant lesion and probably represents severe anal intraepithelial neoplasia (AIN). It is estimated to progress to invasive squamous cell carcinoma (SCC) in 2 to 6% of cases.1,7,8,9,10
Bowen’s disease, like AIN and SCC, is associated with human papillomavirus (HPV) infection types 16 and 18. HPV 16 has been found in 60 to 80% of patients with Bowen’s disease.3 It has been observed that HPV-associated malignancies, including anal in situ cancers, occur more frequently in patients with human immunodeficiency virus (HIV) and the acquired immunodeficiency syndrome (AIDS). Frisch et al examined invasive and in situ HPV-associated cancers among patients with HIV from 5 years prior to the date of AIDS onset to 5 years after onset. The authors found an increased risk of in situ and invasive anogenital cancers in the HIV/AIDS population when compared with the expected number of cancers. When analyzing the 10-year span of AIDS onset, the relative risk (RR) for in situ anal cancers increased significantly from 5 years prior to 5 years after the onset of AIDS. However, the RR for invasive anal cancer changed little over this span, suggesting that immune status did not seem to have as important a role in progression from in situ to invasive malignancy.11
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Bowen’s disease has the highest incidence during the sixth and seventh decades of life. Common symptoms include itching, burning, and occasionally bleeding.1,7,10 Because of the nonspecific nature of complaints, the diagnosis is often made incidentally with pathology obtained for another anorectal procedure. In a study of 33 patients with Bowen’s disease by the Cleveland Clinic, 61% of patients presented with symptoms whereas 39% of individuals were found to have the condition after undergoing a hemorrhoidectomy.12
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Clinically, the lesion often appears as a well-defined erythematous, scaly plaque (Fig. 1). Diagnosis is made by obtaining several full-thickness biopsy specimens from the central portion and edges of the lesion. Histologically, the lesion demonstrates epithelial hyperkeratosis, atypical epithelial cells with mitotic figures and loss of polarity, and full-thickness epidermal involvement. The characteristic finding is the Bowen cell, which appears as a large, atypical cell with a haloed, hyperchromatic nucleus. These cells are periodic acid-Schiff (PAS) negative.1,6,10
Wide local excision has been the treatment of choice in patients with Bowen’s disease, despite the relatively high rate of recurrence.12,13,14 Marchesa et al retrospectively compared patients who had undergone wide local excision versus those with negative microscopic margins to conservative treatment (local excision with gross macroscopic margins and CO2 laser vaporization) and found a significantly lower recurrence rate in the wide local excision group (53.3% versus 23.1%). However, the recurrence rate in the wide local excision group was still 23.1%.14 Because of the inability to determine the extent of disease by gross inspection, frozen section analysis is needed to ensure negative margins. Mapping has been advocated as an effective way to determine the extent of disease.12 Mapping consists of performing a systematic four-quadrant biopsy of the anal canal, anal verge, and perianal skin in addition to performing biopsies of any suspicious or abnormal areas. The involved area is widely excised based on the results of the biopsies. A formal mapping procedure does not preclude recurrence.15
Often, extensive excisions are required, resulting in large wounds that may be difficult to close. Treatment for these wounds has included primary closure, healing by secondary intention, staged excision with split-thickness skin grafts, advancement rotation flaps, myocutaneous flaps, and V-Y island flaps. Flaps and skin grafts have been used primarily in the treatment of large wounds.16
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Because of the need for wide local excisions with large wounds and the relative low rate of developing an invasive SCC, less aggressive treatment options have been used. These more conservative treatment regimens that have been described have included cryotherapy, argon laser therapy, photodynamic therapy, radiation, 5-fluorouracil cream, infrared coagulation, and close observation.17,18,19,20,21,22 A recent study reported good outcomes with the use of 5% 5-fluorouracil treatment in patients with extensive perianal Bowen’s disease.23 Currently, wide local excision remains the treatment of choice.
Follow-up in patients for Bowen’s disease remains unclear. Generally, it has been recommended that patients undergo annual physical examination, proctosigmoidoscopy, and biopsies of prior resection margins and any new lesions. If recurrent disease is found, wide local excision is again the treatment of choice. Patients should be observed for longer than 5 years because of the possibility of late recurrences.14
There has been considerable controversy over whether Bowen’s disease is associated with a risk of developing internal malignancies. Initial reports indicated that there was an association; however, more recent studies have indicated that that seems not to be the case.24,25,26 In a Danish, population-based cohort study of 1147 patients, the authors did not find that patients with Bowen’s disease were at increased risk for developing internal malignant neoplasms; however, they did confirm earlier reports of the higher incidence of nonmelanoma skin cancers among patients with Bowen’s disease.25
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