Extramammary Paget’s disease (EPD) is a cutaneous adenocarcinoma. It is usually curable by wide local excision. However, the prognosis for EPD patients with metastases is extremely poor because effective chemotherapy for advanced EPD has not been established.

Methods

We retrospectively analyzed the efficacy of combination chemotherapy consisting of infusions of 5-fluorouracil (5-FU, 600 mg/m2/body, 5 days/week) and cisplatin (5–10 mg/body, 5–7 days/week) administered intravenously for 8–24 h (low-dose FP).

Results

The patients were 15 males and 7 females and the ages ranged from 54 to 91 years old (median 71). The toxicities of low-dose FP were as follows: hematopoietic dysfunction (n = 4), gastrointestinal dysfunction (4), nephropathy (1), and phlebitis (1). Almost all toxicities were grade 1 or 2 except for 2 cases with grade 3 leukopenia or pancytopenia. Seventeen patients were treated with low-dose FP only, and the clinical results included 10 partial responses (PR), 4 stable disease (SD), and 3 progressive disease (PD). The overall survival ranges (medians) were 5–51 months (12) in all 22 patients, 6–51 months (13) in the 13 patients showing complete response or PR, and 5–12 months (11) in the 6 SD patients. The reported palliative effects of low-dose FP include control of pain and improvement of lymphedema.

Conclusion

Although the number of cases is limited and there is a bias because cases without clinical effects are less likely to be reported, this regimen might be considered a relatively effective option for advanced EPD.