A case of chronic and relapsing paget disease of the vulva

Abstract Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.

A case of microinvasive Paget's disease of the vulva: Treatment of positive resection margin with topical 5-FU cream application / 대한산부인과학회지

Extramammary Paget's disease of the vulva is a rare lesion that accounts for 1~2% of vulvar neoplasms. Paget's disease often has a microscopic extension beyond the gross lesion and shows a multifocal distribution. Positive resection margin is common. We applied 5-FU cream on the lesion who had a positive resection margin. There was no residual lesion on multiple punch biopsies after 5-FU cream treatment three months later. And there has been no evidence of disease recurrence in the 12-month follow-up period. We experienced a case of effective treatment with 5-FU cream in microinvasive Paget's disease of the vulva with positive resection margin. We present it with a brief review of literatures.

A Case of Vulvar Paget's Disease Associated with Adenocarcinoma Occurred in Young Woman / 대한산부인과학회잡지

Extramammary Pagets disease of the vulva is an uncommon vulvar neoplasm with a high rate of recurrence. We report a young woman in whom vulval Paget's disease occurred in association with local adenocarcinoma. The patient presented at the age of 28 years with slowly growing vulval mass which had some mucoid discharge. Biopsy of the mass showed Paget's disease with associated local adenocarcinoma. This patient received modified radical vulvectomy with unilateral inguinofemoral lymphadenectomy. Until now she is under follow up without evidence of recurrence for 24 months. According to our medline research from 1980 to 2000, this case is the youngest asian patient yet recorded. We recommend that abnormally growing vulvar mass need to be biopsied eventhough she is young.