Paget's disease of the vulva: Diagnosis and follow-up key to management; a retrospective study of 50 cases from Queensland

Abstract

Objective: To review the clinical features, diagnosis, management, and outcomes for the 50 cases of Paget's disease (PD) of the vulva referred to Queensland Centre for Gynaecological Cancer between 1986 and 2009.

Methods: Vulvar PD cases from QCGC were reviewed and analyzed using the computer software Statistical Package for the Social Sciences (SPSS) 11.0.

Results: Paget's disease (PD) of the vulva is uncommon. Of the 50 patients, 2 have died of their PD, 1 patient that had coexisting PD died of squamous cell vulva cancer, and 11 died of unrelated causes. The mean age at diagnosis was 67.6 years (range, 31 to 91). All cases were Caucasian. Time from onset of symptoms to diagnosis averaged 21 months. Not until a biopsy was performed was the diagnosis made. The most common presenting complaint was pruritis (27 cases, 54%). There was no identifiable "favored" site on the vulva for PD. Positive groin lymph nodes were found in 4 of the 10 cases who underwent node biopsy. Two who had poorly differentiated carcinoma in the nodes and PD died of disease within a year of diagnosis, one is alive three years later. The fourth case had coincidental PD and vulvar squamous cell carcinoma with squamous carcinoma groin nodes. Initial treatment was surgical.

Conclusions: The prognosis for primary extra-mammary PD of the vulva confined to the epidermis (IEP) is excellent. Early diagnosis and long term follow-up are the keys to successful management. The status of disease at the margins of surgical specimens does not reliably equate to patient long term outcomes.