Penoscrotal Extramammary Paget???s Disease: A Review of 33 Cases in a 20-Year Experience

Abstract

Extramammary Paget's disease in men most frequently involves the penoscrotal area. The uncertainty of the outcome and of the relationship to the underlying adnexal carcinoma and associated internal malignancy still exists. From 1982 to 2001, 33 patients with penoscrotal extramammary Paget's disease were treated and followed up. Therapeutic modalities included carbon dioxide laser ablation (two patients) and local wide excision (31 patients). Split-thickness skin graft (22 patients), local scrotal flap (six patients), and primary closure (three patients) were utilized to reconstruct the penoscrotal defects after local wide excision. An underlying adnexal carcinoma occurred in seven of 33 patients (21.2 percent). The incidence of associated internal malignancy was 9.1 percent (three of 33 patients), including one concurrently and two nonconcurrently associated malignancies. Eight of 33 patients had local recurrence, representing an incidence of 24.2 percent. Three patients (9.1 percent) had distant metastasis and ultimately died of metastatic carcinoma. Of these patients, 31 were grouped according to the degrees of involvement: limited to the epidermis (group 1, n = 14), involvement of the adnexal gland and/or hair follicle (group 2, n = 10), and the presence of an underlying adnexal carcinoma (group 3, n = 7). Local wide excision with subsequent reconstruction by split-thickness skin graft was favored in this series. Patients with an underlying adnexal carcinoma or pathological invasion of the dermis (group 2 or 3) had a worse prognosis than patients without. From this study, it is difficult to address the particular relationship between the outcome and the associated internal malignancy.

Management of Penoscrotal Extramammary Paget Disease: Case Series and Review of the Literature

“…Invasive genitourinary tract cancers (prostate, bladder, kidney) [22][23][24][25][26][27][28] or gastrointestinal sites (colon, rectum, liver) 29,30 can be associated, synchronously or metachronously, with penoscrotal empd, but the frequency of this association is not consistently reported. In a recent M.D.…”

Reconstruction with scrotal skin flaps after wide local resection of penoscrotal extramammary Paget's disease

“…In general, wide local resection might be the best choice for EMPD because it offers the possibility of a cure [ 1 ] . The prevalence of local recurrence after wide local resection of penoscrotal EMPD has been reported to be 19.4 -27.3% of patients [ 3,4,7 ] . Early studies by our research team indicated that frozen section-guided wide local resection produces an acceptable prevalence of recurrence (16%) when treating penoscrotal EMPD.…”

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

“…9,11,14 In this report, we introduce the 'anterolateral thigh perforator flap' technique to reconstruct defects after the resection of penoscrotal EMPD lesions. The anterolateral thigh flap is an effective option for the reconstruction of soft-tissue defects such as those in the head, neck, extremities, perineal and scrotal areas.…”

“…The primary treatment is surgical excision. 7,9,11,[14][15][16][17] Non-surgical methods are also used but only in a few cases. Hata reported that radiotherapy was a curative therapy in 22 patients with EMPD.…”

“…[4][5][6][7][8] However, local wide excision and immediate reconstruction is the preferred treatment of Chinese urologists. [9][10][11] Our experience with 130 patients evaluated at mid-term follow-up demonstrated that intraoperative frozen-section biopsy was a reliable means to detect invisible tumours and could significantly reduce recurrence. 3 When we used this technique to completely resect the lesions and preserve normal tissues, we encountered the challenge of reconstructing the different sizes and shapes of postoperative skin defects ( Figures 1b, 2b, 3b and 4b).…”