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Paget's disease of the vulva
J P Curtin1, S C Rubin, W B Jones
1Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021.
Gynecologic Oncology
|December 1, 1990
Summary
Surgical treatment for Paget's disease of the vulva is effective, with most patients achieving disease-free status. Early diagnosis and appropriate surgical intervention, including local excision for superficial cases, are crucial for successful outcomes.
Area of Science:
- Gynecologic Oncology
- Dermatopathology
Background:
- Paget's disease of the vulva is a rare condition affecting older women.
- It can occur as superficial noninvasive disease or be associated with invasive adenocarcinoma.
Purpose of the Study:
- To review treatment outcomes for Paget's disease of the vulva.
- To evaluate the efficacy of surgical management for both superficial and invasive forms.
Main Methods:
- Retrospective review of 36 patients diagnosed with Paget's disease of the vulva.
- Analysis of treatment modalities, including surgical procedures and follow-up data.
- Correlation of treatment with disease recurrence and survival rates.
Main Results:
- 14% of patients had associated invasive adenocarcinoma.
- For superficial Paget's disease, 22 of 28 patients remained disease-free after surgery.
- Six patients experienced recurrence requiring multiple procedures.
- Radical surgery offered long-term survival for patients with invasive adenocarcinoma.
Conclusions:
- Surgical management is the primary treatment for Paget's disease of the vulva.
- Local excision with frozen-section margin evaluation is recommended for superficial disease.
- Aggressive surgical approaches are necessary for invasive adenocarcinoma, with favorable survival observed in some cases.