From the Guidelines
Paget's disease of the anus is a rare type of intraepithelial adenocarcinoma that requires prompt medical attention and typically surgical management, with wide local excision being the primary treatment approach. The diagnosis of Paget's disease is made through biopsy, which shows characteristic Paget cells with abundant pale cytoplasm and large nuclei 1.
Treatment Approach
The treatment approach for Paget's disease of the anus involves a multidisciplinary approach, including physicians from gastroenterology, medical oncology, surgical oncology, radiation oncology, and radiology 1. For small, localized lesions without invasive disease, wide local excision may be sufficient. However, if there is underlying invasive adenocarcinoma or extensive disease, more aggressive surgery such as abdominoperineal resection may be necessary.
Preoperative Evaluation
Patients should undergo thorough preoperative evaluation, including:
- Colonoscopy
- Anorectal examination
- Imaging studies (MRI or endorectal ultrasound) to assess the extent of disease and identify any underlying malignancy, as Paget's disease is often associated with concurrent adenocarcinoma 1.
Follow-up and Adjuvant Treatment
Following surgery, regular follow-up is essential due to high recurrence rates, typically every 3-6 months for the first two years. Radiation therapy may be used as adjuvant treatment in cases with positive margins or invasive disease. Topical treatments like imiquimod 5% cream or 5-fluorouracil may be considered for patients who are poor surgical candidates, though these are less effective than surgical approaches.
Disease Presentation and Diagnosis
The disease presents as a red, scaly, pruritic lesion around the anus and is diagnosed through biopsy showing characteristic Paget cells with abundant pale cytoplasm and large nuclei. The NCCN Anal Carcinoma Guidelines Panel recommends a multidisciplinary approach for treating patients with anal carcinoma, which includes Paget's disease of the anus 1.
From the Research
Diagnosis of Paget's Disease of the Anus
- The diagnosis of Paget's disease of the anus is often delayed due to nonspecific symptoms such as pruritus and perianal bleeding 2
- Histopathologic demonstration of Paget's cells from a biopsy is necessary to confirm the diagnosis 3
- Immunohistochemical studies can help determine the origin of the Paget's cells and identify potential markers for malignancy 4
Treatment of Paget's Disease of the Anus
- Wide local excision is the treatment of choice for non-invasive Paget's disease of the anus 4, 3, 2
- For invasive disease, abdominoperineal resection may be necessary 2
- Topical imiquimod therapy has been shown to be effective in some cases of perianal extramammary Paget disease 5, 6
- Reconstruction with gluteal skin flap may be necessary after wide excision 3
Prognosis and Follow-up
- Perianal Paget's disease is associated with a high risk of local recurrence and potential metastasis to distant sites 4, 2
- Long-term follow-up is necessary to monitor for recurrence and metastasis, including periodic proctoscopy, digital examination, and imaging of liver and lungs 2
- The prognosis is generally poor when underlying invasive carcinoma is present 3