Related Experiment Videos
Extramammary Paget's disease with aggressive behavior: a report of two cases
1Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea. jckim@www.amc.seoul.kr
Abstract:
Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disorder which is included as a rare malignant condition. However, it sometimes shows aggressive behavior of local recurrence and coexisting malignancy. We had experienced nine cases of EMPD involving the scrotum for seven years. Two cases of them presented metastasis. The first case presented extensive inguinal lymph node metastasis with underlying adnexal adenocarcinoma one year after wide local excision. The second case initially presented multiple metastasis to the liver and in the lymph node. The latter, showing fulminant progression with liver metastasis, may be only the second case reported in English literature. EMPD is considered as a malignant neoplasm with aggressive behavior from initial presentation. Because wide local excision of the lesion alone may be occasionally insufficient, a careful follow-up must be done to detect recurrence or internal malignancy.
Insights
Extramammary Paget's disease (EMPD) can be aggressive, with rare scrotal cases showing metastasis. Careful follow-up is crucial for detecting recurrence or coexisting malignancy after treatment.
Area of Science:
- Oncology
- Dermatology
Background:
- Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm.
- It can exhibit aggressive behavior, including local recurrence and coexisting malignancies.
Observation:
- A review of nine scrotal EMPD cases over seven years identified two instances of metastasis.
- One case involved extensive inguinal lymph node metastasis with underlying adnexal adenocarcinoma.
- The second case presented with rapid progression and multiple liver and lymph node metastases, a rare occurrence.
Findings:
- EMPD, particularly in the scrotum, should be considered a malignant neoplasm with potentially aggressive behavior.
- Wide local excision alone may not be sufficient for complete disease management.
Implications:
- Scrotal EMPD necessitates vigilant follow-up protocols.
- Early detection of recurrence or internal malignancy is critical for improved patient outcomes.