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Extramammary Paget's disease of the perianal region
H Tulchinsky1, O Zmora, E Brazowski
1Department of Surgery B, Tel-Aviv Sourasky Medical Centre, Tel-Aviv University, Tel Aviv, Israel. hamtul@hotmail.com
Objective:
Perianal Paget's disease (PPD) is a rare entity. The standard treatment for either in situ or invasive extra mammary Paget's disease (EMPD) is surgical excision. Local recurrence and morbidity from surgery, especially in the elderly, can, however, be high. The aim of this article is to review our experience with PPD and question the currently preferred treatment approaches in light of its histopathology and therapeutic outcome.
Patients And Methods:
A chart review of our patients with PPD from 1996 to 2002 was carried out to determine their outcome after treatment. Data from review of the literature are presented.
Results:
Five patients with in situ disease (four females, median age 68 years) were diagnosed as having PPD. A complete surgical excision was attempted in 4 patients and the fifth was treated by photodynamic therapy. At present, all patients are alive, two are free of disease, one has persistent disease and two have local recurrence.
Conclusion:
Considering the significant rate of recurrence even after wide local excision, the extent of surgery needed and the good prognosis with long-term survival, we question whether nonsurgical modalities should be considered in place of surgery as primary treatment for noninvasive PPD, with radical surgery being reserved for failures or invasive disease.
Insights
Perianal Paget's disease (PPD) treatment outcomes show significant recurrence rates after surgery. Non-surgical options may be considered for non-invasive PPD, reserving radical surgery for invasive disease or treatment failures.
Area of Science:
- Dermatology
- Surgical Oncology
- Oncology
Background:
- Perianal Paget's disease (PPD) is a rare condition.
- Surgical excision is the standard treatment for PPD.
- High recurrence rates and morbidity, especially in elderly patients, pose challenges.
Purpose of the Study:
- To review institutional experience with PPD.
- To evaluate treatment outcomes for PPD.
- To question current surgical treatment paradigms for PPD based on histopathology and outcomes.
Main Methods:
- Retrospective chart review of PPD patients (1996-2002).
- Analysis of treatment outcomes.
- Literature review for comparative data.
Main Results:
- Five patients with in situ PPD (median age 68) were analyzed.
- Four patients underwent surgical excision; one received photodynamic therapy.
- Outcomes included two disease-free, one persistent disease, and two local recurrences.
Conclusions:
- Significant recurrence rates observed even after wide local excision.
- Non-surgical modalities warrant consideration as primary treatment for non-invasive PPD.
- Radical surgery should be reserved for treatment failures or invasive disease.
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