Surgical Treatment of Penoscrotal Extramammary Paget's Disease

陰莖陰囊部位之乳房外柏哲德氏病的手術治療

10.7096/TJTSPS.201003.0033

曾元生(Yuan-Sheng Tzeng)；陳錫根(Shyi-Gen Chen)

Extramammary Paget's disease EMPD ； penis ； scrotum ； Bowen's disease

臺灣整形外科醫學會雜誌

19卷1期（2010 / 03 / 01）

33 - 39

英文

背景： 陰莖陰囊部位之乳房外柏哲德氏病相當罕見，大多只有零星的案例報告。 目的： 我們報告了二十五年來一共八例的手術處置並做分析與探討。 材料與方法： 八位病患均做了廣泛性的切除並依切除範圍大小選擇做直接縫合或皮瓣覆蓋。 結果： 總共有一位病患復發，兩位病患死亡，五位病患治癒。 結論： 從我們的結果得知，兩公分的廣泛性切除是足夠的，但若病患合併有鼠蹊部位淋巴結的轉移，則有較差的預後。

Aims: Extramammary Paget's disease (EMPD) of the penis and scrotum is extremely rare and only reported sporadically. We describe the distinct manifestations of penoscrotal EMPD; possible differential diagnosis; treatment modalities with focused on the surgical principle and reconstruction options; and oncologic outcome based on our 25 years experiences. Material and Methods: Between 1982 and 2006; 8 patients with penoscrotal EMPD underwent wide local excision of the disease. The charts of the resultant 8 cases were reviewed for demographic and clinical data; including age at presentation; disease location; associated internal malignancy; treatment; reconstruction method; and evidence of recurrence or metastasis. Results: In our 8 patients; scrotal involvement was presented in 2 patients; penile involvement was presented in 1 patient and penoscrotal junction was presented in 5 patients. Superficial inguinal nodes were involved in 2 cases. None of our patients had associated internal malignancy. At a mean follow-up of 8 years (range 1 to 15 years); adequate cosmetic and functional results were obtained. Recurrence of EMPD has developed in one patient 2 years after first excision; and resolved by another wide excision and skin grafted. Two patients with inguinal lymphadenopathy died from pulmonary metastasis. Five patients were free of disease. Conclusion: Wide local excision of the involved lesion with at least 2 cm safe margin and under the guidance of frozen section; hen reconstruction with primary closure or expanding remnant flap provided adequate cosmetic and functional results and acceptable survival. Disease may spread to the regional lymph nodes and patients with lymphadenopathy had a worse prognosis.