Extramammary Paget disease of the vulva: minimal excision with adjuvant radiation treatment for optimal aesthetic results.

AIMS AND BACKGROUND: Extramammary Paget disease (EMPD) is an uncommon malignant neoplasm that occurs in areas containing apocrine glands, and the vulva is the most commonly involved site. Wide surgical excision with subsequent defect reconstruction is widely accepted as the first-line treatment for EMPD. More recently, radiation treatment has been considered an appropriate primary or adjuvant treatment. In an effort to replace wide excision and avoid unfavorable aesthetic results and sexual dysfunction, we decided to excise the tumor minimally and to add adjuvant radiation treatment.

METHODS: A 71-year-old woman had EMPD at the right labium majus. The patient was treated by minimal excision with a 1-cm safety margin and by adjuvant radiation treatment weekly from 1 month to 3 months after surgery to a total dose of 70.2 Gy. Four months after completing the radiation treatment, a 10-point surgical biopsy around the site of previous resection was performed. Radiation treatment caused hypertrophy of the left labium minus, which was treated by additional labiaplasty (labium minus reduction) to obtain optimal aesthetic results.

RESULTS: Surgical biopsy revealed no definite evidence of recurrence. Currently, the patient had no EMPD symptoms, is satisfied with a symmetrical vagina, and experiences no discomfort during ordinary activities or intercourse.

CONCLUSIONS: Minimal tumor excision (with a 1-cm safety margin), adjuvant radiotherapy, and additional labiaplasty were performed to treat EMPD. Complete tumor removal without recurrence, an aesthetically satisfactory result, and normal sexual function were achieved in this patient.