Recent treatment and prognosis in 643 patients with extramammary Paget's disease

Area of Science:

• Oncology
• Dermatology

Background:

• Extramammary Paget's disease (EMPD) is rare, leading to limited treatment data.
• Prognosis varies significantly between in situ and invasive EMPD, necessitating stage-based treatment planning.

Purpose of the Study:

• To analyze recent trends in extramammary Paget's disease treatment and prognosis.
• To evaluate the efficacy of the EMPD-oriented TNM staging system.

Main Methods:

• Retrospective analysis of 643 EMPD cases treated in Japan (2015-2019).
• Assessment of treatment patterns and survival outcomes stratified by TNM stage.
• Multivariate analysis to identify prognostic factors.

Main Results:

• Survival curves differed by stage, except for stages II and IIIA.
• Curative surgery was standard for stages 0-III; chemotherapy (docetaxel-based regimens) was primary for stages IIIB-IV.
• Female sex predicted local relapse in early stages; curative surgery improved survival in advanced stages.

Conclusions:

• Treatment and prognosis for extramammary Paget's disease are stage-dependent.
• Further research is crucial to enhance survival for patients with advanced-stage EMPD (stages II-IV).