Korean J Dermatol.  2022 Jul;60(6):343-349.

Analysis of Treatment Methods and Prognosis for Patients with Extramammary Paget’s Disease for 20 Years at a Single Tertiary Medical Institution: Carcinoembryonic Antigen as a Risk Factor for Metastasis

• Park JH
• Lee JB
• Yun SJ

Abstract

Background
Extramammary Paget’s disease (EMPD) is a rare cutaneous malignancy of anogenital lesions.
Objective
To investigate the long-term prognosis of patients with EMPD and the results of various treatment methods for 20 years.
Methods
We retrospectively reviewed the medical records of 80 patients with biopsy-proven EMPD at Chonnam National University Hospital during the last 20 years from 2000 to 2019.
Results
The most common treatment option was excision in male patients (75.0%). However, for female patients, nonsurgical treatments, such as topical imiquimod (29.1%) or radiation therapy (20.8%), were mostly applied. In the long-term follow-up, 10 patients (12.5%) were diagnosed with systemic metastasis. The most common metastatic site was the lymph nodes, followed by the bone. Among patients with metastasis, 66.7% showed elevated serum carcinoembryonic antigen (CEA) levels, whereas none of the non-metastatic patients showed elevated CEA levels. Negative surgical margins were not associated with systemic metastasis but were associated with local recurrence.
Conclusion
Systemic metastasis of EMPD is significantly associated with serum CEA levels. Therefore, more rigorous follow-up, including imaging studies, is required for patients with a poor prognosis.

Keyword

Carcinoembryonic antigen; Extramammary Paget disease; Neoplasm metastasis