Extramammary Paget disease of the vulva: minimal excision with adjuvant radiation treatment for optimal aesthetic results.
OBJECTIVE
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.
Department of Plastic and Reconstructive Surgery, Dongguk University College of Medicine, Gyeongju - Republic of Korea.
Myeong Su
MS
Jeon
Department of Plastic and Reconstructive Surgery, Dongguk University College of Medicine, Gyeongju - Republic of Korea.
Gyu Yong
GY
Jung
Department of Plastic and Reconstructive Surgery, Dongguk University College of Medicine, Gyeongju - Republic of Korea.
Joon Ho
JH
Lee
Department of Plastic and Reconstructive Surgery, Dongguk University College of Medicine, Gyeongju - Republic of Korea.
Kyung Won
KW
Kang
Department of Radiation Oncology, Sunlin Medical Center, Pohang - Republic of Korea.
Kwang Won
KW
No
D000368:Aged; D001706:Biopsy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D010145:Paget Disease, Extramammary / Q000175:diagnosis / Q000532:radiotherapy* / Q000601:surgery*; D017060:Patient Satisfaction; D018714:Radiotherapy, Adjuvant; D016896:Treatment Outcome; D014846:Vulvar Neoplasms / Q000175:diagnosis / Q000532:radiotherapy* / Q000601:surgery*