Clinical treatment of vulvar Paget's disease: a case report

Abstract

Vulvar Paget's disease is very rare malignancy originating in vulvar apocrine-gland-bearing skin cells or as a manifestation of adjacent primary anal, rectal or bladder adenocarcinoma.1 It is also called extramammary Paget's disease and affects both sexes, in the vulvar, perineal or scrotal areas. Vulvar Paget's disease is most commonly seen in postmenopausal Caucasian females and appears clinically as red, eczematous and pruriginous – and sometimes painful – lesions. In most cases it is an intraepidermal adenocarcinoma in which tumor cells involve the epidermis and sometimes the underlying skin appendage, but sometimes there is focal dermal invasion.2 Probably because of its multicentric nature, Paget's disease has a chronic and relapsing course. Surgery has been the treatment of choice, but disease frequently extends far from the visible lesion and surgical margins are frequently positive. The Paget's cells are larger than keratinocytes, have clear chromatin, a prominent nucleolus and gray–blue cytoplasm and they may appear vacuolated with hematoxylin and eosin staining.3 All these characteristics are consistent with their glandular nature. Many patients undergo multiple surgical procedures, including wide local excision, simple vulvectomy or radical vulvectomy. However, the margins of primary surgical specimens are positive in more than half of the patients.3 Consequently, the recurrence rate is about 47%.4 The disappointing results of surgical treatment and the high morbidity and side-effects of extensive vulvar resection have encouraged many authors to try innovative clinical treatment for vulvar Paget's disease.