Penoscrotal extramammary Paget's disease: surgical techniques and follow-up experiences with thirty patients

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.

Figure 1

The frozen biopsy was prepared (a). Scrotal skin was used to cover the wound directly (d and e), or scrotal skin was used as a random flap to cover the wound (b and c).

Figure 2

The diseased region was large (a), and the lesion size increased after resection (b). The wound was minimized by scrotal flaps (c) and covered with split-thickness skin grafts (d).

Figure 3

A frozen biopsy is shown that is positive for malignant tissue at the red points and negative at the green points (a). The lesion was covered by total split-thickness skin grafts (b and c). The result was satisfactory one month after the surgery (d).

Figure 4

One case exhibited a huge wound and an ulcer in the perineum (a). The lesion was both large and deep after resection (b). The lateral femoral vessels were probed by Doppler ultrasound, and an anterolateral thigh perforator flap was designed (c and d). The wound was well-covered by this flap (e) and healed well (f).