Background: Extramammary Paget disease (EMPD) is a cutaneous neoplasm that can metastasise to the lymph nodes and distant organs, resulting in a poor prognosis. For unresectable distant metastases of EMPD, no consensus has been reached regarding the optimal chemotherapy owing to the lack of data.
Objectives: To evaluate the efficacy of three regimens, docetaxel (DTX) monotherapy, combination therapy with 5-fluorouracil, epirubicin, carboplatin, vincristine, and mitomycin C (FECOM), and tegafur (S-1) monotherapy.
Methods: This single-centre retrospective study included 32 patients diagnosed with unresectable EMPD and treated with chemotherapy between 2002 and 2022 at the National Cancer Center Hospital in Japan. Patient characteristics, responses to treatment, and survival data were evaluated for each of the first-line therapies.
Results: Among the 17 patients who received DTX monotherapy, the response rate (RR) and disease control rate (DCR) were 47.1% and 76.5%, respectively. The median progression-free survival (PFS) and overall survival (OS) were 12.3 months (95% confidence interval [CI]: 6.1-26.6) and 19.2 months (95% CI: 8.5-not reached), respectively. Among the 11 patients who received combination FECOM chemotherapy, the RR and DCR were 54.5% and 63.6%, respectively. The median PFS and OS were 6.8 months (95% CI: 3.5-not reached) and 13.4 months (95% CI: 8.6-21.3), respectively. Among the four patients who received S-1 monotherapy, the RR and DCR were 0.0% and 25.0%, respectively. The median PFS and OS were 5.4 months (95% CI: 2.3-not reached) and 12.5 (95% CI: 2.3-not reached) months, respectively.
Conclusions: Further investigations with prospective analysis are required to confirm these findings.
Authors: Shogo Wada, Dai Ogata, Eiji Nakano, Kenjiro Namikawa, Naoya Yamazaki.