- 期刊
Extramammary Paget's Disease-32 Years Experiences in KMUH
乳房外柏哲德氏病－高醫32年之臨床經驗
摘要
背景：乳房外柏哲德氏病並不是一個常見的疾病，且因其搔癢性的紅色斑塊外觀常讓它被誤診為濕疹。此病過去被認為主要發生在女性，另一個為人所知的就是它經常同時伴行有臟器器官或是病灶下方皮膚腺體的惡性腫瘤。近期於亞洲國家所開展的研究顯示出許多東方人與西方人在臨床表現的不同之處。除此之外，居高不下的局部復發率也是值得外科醫師所注意的。目的與目標：此研究的目標主要在統計本院32年來乳房外柏哲德氏病之臨床表現、處理及預後情況。材料及方法：在介於1980年至2011年的32年間，共蒐集了32位患有乳房外柏哲德氏病的病人，我們主要統計這些病人的性別、確診年齡、初始症狀及經過時間、病灶位置、有無伴行之惡性腫瘤、治療方式及預後情況。結果：我們的結果顯示此疾病以男性居多（7:1）。確診年齡平均為66.1歲。所有的病灶均圍繞在生殖器官周圍，以男性中陰莖與陰囊的交界處為最常發生的位置。症狀發生至確診的時間平均為3.8年，在確診前以濕疹為最常出現的診斷，其次為股癬和性病。在我們的統計中發現了三位病人（9.3%）有伴行的惡性腫瘤。病理報告顯示病灶主要侷限在表皮內（74%），僅7位病人（26%)有真皮層的侵犯，系列中無任何皮脂腺惡性腫瘤的發現。此系列中多數的病人（84%）均接受以3公分為安全距離的廣泛慶腫瘤切除並輔以術中冷凍切片來確認腫瘤之清除乾淨。在有追蹤到的所有術後病人中，以平均4.5年時間觀察均無腫瘤局部復發之現象。此系列中亦無發現有遠處轉移或因乳房外柏哲德氏病而死亡的個案。結論：此研究統計了乳房外柏哲德氏病在台灣以男性居多的情況，且比起西方人有著較低的臟器惡行腫瘤及皮脂腺癌伴行率。仔細辨識病灶邊緣對於手術切除的執行非常重要，我們的切除方式採取3公分的安全距離並搭配術中冷凍切片而得到令人滿意的局部復發率。輔以鼠蹊皮瓣則可在恥骨上方之缺損達到不錯的重建效果。
關鍵字
無資料並列摘要
Background: Extramammary Paget's disease (EMPD) is rare and usually mistaken for eczema due to its appearance as an erythematous and pruritic plaque. It is well-known for its female predominance as well as association with the internal malignancy and subjacent adnexal adenocarcinoma. Recent studies in Asian countries started to show many different clinical manifestations between Oriental and Caucasian population. Besides, high local recurrence rate after wide excision is still a notable issue for many surgeons. Aim and Objectives: The aim of this study is to investigate the biologic behavior, management and outcome of EMPD based on our 32 years experiences. Materials and Methods: Between 1980 and 2011, 32 patients with EMPD were reviewed for demographic and clinical data including age at diagnosis, initial presentation, duration of symptoms, location of lesions, presence of associated malignancy, treatment modalities and outcome of disease. Results: The disease exhibited a male predominant (7:1) pattern. The mean age at diagnosis was 66.1 years old. All lesions were around genital organs. The junctions of penis and scrotum were involved in half of male patients. The mean duration of illness before diagnosis was 3.8 years. Eczema was the most common initial diagnosis before biopsy, followed by tinea cruris and venereal disease. Concurrent malignancy was found in 3 patients (9.3%). Histopathologic findings revealed the the lesions confined within epidermis in most of patients (74%). Only 7 patients (26%) showed dermal invasion of Paget's cells and none had concurrent adnexal adenocarcinoma. The majority of our patients (84%) received wide excision with 3cm surgical margin combining intraoperative frozen section as definite treatment. There is no local recurrence for the patients within follow-up for an average of 4.5 years. No distant metastasis or mortality from EMPD was noted in this series. Conclusion: Male predominance of EMPD in Taiwan is documented in this study. Lower incidence of associated internal malignancy and subjacent adnexal adenocarcinoma is also revealed and compatible with other Asian studies. Survey of associated internal malignancy is still suggested for its notable incidence. Combining with intraoperative frozen section, wide excision with 3 cm surgical margin should be performed with careful identification of the tumor border. Pedicled groin flaps can be combined with scrotal flaps to achieve acceptable results especially for the defects extending to suprapubic area.