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Clinical and Pathological Analysis of Perianal Paget's Disease: A Case Report and Review of 89 Cases
Kae Ishii1, Hidekazu Takahashi1, Hiromi Tsuji2
1Department of Gastroenterological Surgery, Osaka International Medical and Science Center, Osaka Keisatsu Hospital, Osaka, Japan.
Background/Aim:
Perianal Paget's disease (PPD) is an intraepithelial invasion of the perianal skin that is frequently associated with anorectal carcinoma. Rectal canal carcinoma with Pagetoid spread (PS) is a relatively rare disease, and few reports on its outcomes are available. The relatively rare nature of this disease makes the development of treatment recommendations difficult. This study aimed to clarify the characteristics of the disease and factors related to prognosis. We present a case report and review of 89 cases, including those from the existing literature and our own experience.
Case Report:
The patient was an 81-year-old man who was referred to our hospital for surgery after endoscopic examination of the lower gastrointestinal tract revealed cancer of the anal canal with PS. Physical examination revealed erythema and erosions around the anus. Lower gastrointestinal endoscopy revealed an erythematous area and a continuous raised lesion in the anal canal. We diagnosed the patient with anal canal carcinoma with PS, and robot-assisted abdominoperineal resection was performed.
Conclusion:
Our findings indicate that the five-year survival rate for PPD patients was 63%, comparable to that of anal canal cancer without PS. The histological type and presence of lymph node metastasis may be related to the prognosis. The study suggests that early-stage patients with favorable histological types and no lymph node involvement may benefit from less invasive treatment options, such as endoscopic submucosal dissection.
Insights
Perianal Paget's disease (PPD) survival is comparable to anal canal cancer. Prognosis depends on histology and lymph node metastasis, suggesting tailored treatments for PPD.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Dermatology
Background:
- Perianal Paget's disease (PPD) is an intraepithelial neoplasm of the perianal skin.
- It is often associated with underlying anorectal carcinoma.
- Rectal canal carcinoma with Pagetoid spread (PS) is rare, complicating treatment recommendations.
Purpose of the Study:
- To clarify the characteristics of perianal Paget's disease with Pagetoid spread.
- To identify factors influencing prognosis in these patients.
- To review existing literature and present a case study.
Main Methods:
- A comprehensive literature review was conducted.
- Data from 89 cases, including a new case report, were analyzed.
- Statistical analysis focused on survival rates and prognostic factors.
Main Results:
- The five-year survival rate for PPD patients was 63%, similar to anal canal cancer without PS.
- Histological type and lymph node metastasis were identified as potential prognostic indicators.
- A case of anal canal carcinoma with PS treated with robot-assisted abdominoperineal resection is presented.
Conclusions:
- Perianal Paget's disease shares similar survival outcomes with anal canal cancer.
- Histological features and lymph node status are crucial for predicting prognosis.
- Early-stage PPD with favorable characteristics may be amenable to less invasive treatments like endoscopic submucosal dissection.
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