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 64.9629.6 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.

Extramammary Paget′s disease: Analysis of 17 Chinese cases

“…Poor prognostic factors include dermal invasion or metastasis via the lymphatic system. [7,29] Lymph node metastasis in most cases involves inguinal, peri-rectal, retroperitoneal, iliac, and para-aortic lymph nodes. Distant metastasis involves predominantly the liver, bone, lung, and suprarenal glands.…”

Tumor Wide Horizontal Invasion Predicts Local Recurrence for Scrotal Extramammary Paget’s Disease

“…Some studies demonstrated that clear margins were highly relevant to whether local recurrence occurs 20 . Therefore major techniques including Mohs micrographic surgery, fluorescent dyes and frozen biopsy were extensively applied to defining the actual margin during surgery 25 . Although these methods could eventually ensure the clear margins, local recurrence persisted.…”

Anatomic Subtype Differences in Extramammary Paget Disease

“…; eFigure in Supplement 1), and 135 articles were retained when (1) cases were limited to a single anatomic subtype (ie, perianal, vulvar, or penoscrotal) or (2) case-level data allowed distinction among these 3 anatomic subtypes (eTable in Supplement 1). Among the included articles, 113 (83%) included 10 or more cases of EMPD, suggesting reasonable cohort sizes considering the rarity of the disease. Cases that were metastatic on presentation were excluded.…”