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Penoscrotal extramammary Paget's disease: surgical techniques and follow-up experiences with thirty patients
Qi Chen1, Yan-Bo Chen, Zhong Wang
1Department of Urology and Andrology, Ninth People's Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai 200011, China.
Abstract:
To report the surgical management, complications and prognosis of patients with penoscrotal extramammary Paget's disease (EMPD) at different clinical stages. Between 2003 and 2008, a total of 30 male patients with penoscrotal EMPD were enrolled and evaluated. All enrolled subjects received frozen biopsy-guided local wide resection and immediate reconstruction. Patients were followed every 3 months postoperatively. Among the 30 patients who accepted and underwent frozen biopsy-guided local wide resection treatment and reconstruction, two (6.7%) cases exhibited positive margins, verified by pathological examination, and underwent re-excision after surgery. The technique of primary closure or an adjacent flap was used in 10 (33.3%) cases, split-thickness skin grafts were used in 15 (50%), and an anterolateral thigh perforator flap was used in five cases (16.7%). The postoperative complications were acceptable. The mean follow-up time was 64.9 ± 29.6 months. Of all 30 cases, 22 patients (73.3%) survived with no evidence of recurrence, four patients (13.3%) exhibited local recurrence, two patients (6.7%) exhibited both local recurrence and distant metastasis and the remaining two patients (6.7%) exhibited distant metastasis. Five patients died from metastasis or cachexia. Current surgical techniques, including primary closure, adjacent flaps, split-thickness skin flaps and anterolateral thigh perforator flaps are able to reconstruct all types of defects with acceptable complications. Some patients with negative margins went on to exhibit local recurrence, potentially due to adnexal carcinoma or internal malignancy.
Insights
Surgical management of penoscrotal extramammary Paget's disease (EMPD) involves wide resection and reconstruction. While complications are acceptable, recurrence remains a concern, highlighting the need for vigilant follow-up.
Area of Science:
- Urology
- Surgical Oncology
- Dermatopathology
Background:
- Extramammary Paget's disease (EMPD) is a rare malignancy affecting the external genitalia.
- Penoscrotal EMPD presents unique surgical challenges and requires careful management.
- Understanding surgical outcomes and prognosis is crucial for patient care.
Purpose of the Study:
- To evaluate surgical management, complications, and prognosis of penoscrotal EMPD.
- To analyze outcomes based on different clinical stages and surgical techniques.
- To identify factors influencing recurrence and survival rates.
Main Methods:
- Retrospective analysis of 30 male patients with penoscrotal EMPD (2003-2008).
- Frozen biopsy-guided local wide resection and immediate reconstruction.
- Follow-up every 3 months, utilizing various reconstruction techniques (primary closure, flaps, skin grafts).
Main Results:
- Reconstruction achieved with acceptable complications using diverse techniques.
- Local recurrence observed in 13.3%, distant metastasis in 6.7%, and combined in 6.7%.
- Disease-free survival was 73.3%, with 6.7% mortality due to metastasis.
Conclusions:
- Current surgical techniques effectively manage penoscrotal EMPD defects.
- Despite negative margins, recurrence can occur, possibly linked to underlying adnexal or internal malignancy.
- Long-term surveillance is essential for patients with penoscrotal EMPD.
