Invasive extramammary Paget’s disease (EMPD) of the vulva, first identified in 1901, typically affects Caucasians aged 50–65. Approximately 65% of EMPD cases occur in the vulva, representing 1–6% of vulvar cancers in the U.S., with an incidence of 0.36 per 100,000 person-years. Metastasis to lymph nodes worsens prognosis, and 44% of Japanese patients with regional lymph node metastasis develop distant metastasis. Due to the disease's rarity, high-quality interventional or outcomes data are scarce, and randomized clinical trials are unlikely. Treatment for metastatic EMPD is based on retrospective case reports or small series, with varying regimens including monotherapy (docetaxel, cisplatin, 5-FU) and multi-agent chemotherapy (5-FU/cisplatin, cisplatin/epirubicin/paclitaxel). Specific treatment details and efficacy for metastatic vulvar EMPD are lacking. This report highlights a case where monthly low-dose docetaxel achieved a highly effective, durable response with minimal toxicity.
The Outcome of Chemotherapy for Metastatic Extramammary Paget’s Disease
The efficacy and survival impact of conventional chemotherapies for metastatic extra- mammary Paget’s disease (EMPD) have not been fully elucidated. This study examined the long- term outcome of chemotherapy for this indication. We conducted a retrospective review of 21 pa- tients with distant metastatic EMPD (14 patients treated with chemotherapy and 7 patients treated without chemotherapy). The response rate of chemotherapy and patient survival were statistically analyzed.
Successful treatment of metastatic extramammary Paget's disease with pemetrexed monotherapy systemically and 5-fluorouracil topically
Abstract
Advanced extramammary Paget's disease does not have a standardized treatment guideline as its incidence is low and has been rarely reported in literature. Here we describe a case of metastatic extramammary Paget's disease successfully treated with topical 5-fluorouracil (5-FU) and systemic pemetrexed. The therapy was safe without any appreciable adverse effects like diarrhea, rash, neutropenia or fatigue; maintaining remission for more than 6 months. Thus, we propose 5-FU and pemetrexed as the first-line therapy for advanced extramammary Paget's disease, especially for aged patients with unresectable skin lesions.