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題 名 | Penoscrotal Extramammary Paget's Disease with Associated Malignancy--Clinical Experience at Taipei Veteran General Hospital=男性陰莖陰囊部位之乳房外柏哲德氏病合併癌症之研究--臺北榮民總醫院的臨床經驗 |
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作 者 | 陳梅君; 葉發來; 馬旭; 廖文傑; | 書刊名 | 臺灣整形外科醫學會雜誌 |
卷 期 | 22:2 2013.06[民102.06] |
頁 次 | 頁85-96 |
分類號 | 415.78 |
關鍵詞 | 乳房外柏哲德氏病; 癌症; 陰莖; 陰囊; 淋巴結; Extramammary Paget's disease; Penis; Scrotum; Lymph node; Malignancy; |
語 文 | 英文(English) |
中文摘要 | 背景:乳房外柏哲德氏病(EMPD)是一種罕見的表皮腺癌且可能合併潛在癌症。好發在陰唇、肛門直腸及陰莖陰囊處。關於EMPD合併癌症的治療及預後目前仍未有詳細的研究。目的及目標:藉由台北榮民總醫院近25年來治療EMPD合併癌症之病人的經驗,研究合併不同種類的癌症對病人的預後及治療計畫之影響。材料及方法:自1985年1月至2011年12月,共14位病人被診斷為陰莖陰囊處EMPD合併癌症。根據所合併癌症的類形及診斷的時間點,將病人分為三組:合併遲發性癌性、合併皮膚侵犯性腺癌、同時合併其它癌症。經由病歷回顧,整理並分析病人的病史、症狀、合併癌症的類形及時間、腹股溝淋巴結轉移、治療及預後。結果:共59位病人診斷為陰莖陰囊處EMPD,其中4位合併皮膚侵犯性癌症,11位合併其它部位癌症,以前列腺癌最常見(4位,36.4%)。遲發性癌症的平均診斷時間在診斷EMPD後101個月。在同時合併癌症的組別,平均在診斷癌症後82.25個月另外又發現陰莖陰囊處EMPD。遲發性癌症這一組5年存活率為100%。3位合併皮膚侵犯性腺癌的病人皆有腹股溝淋巴結轉移。結論:局部廣泛性切除是目前的標準治療。罹患EMPD的病人,要想到有合併潛在性癌症的可能性,尤其是泌尿道系統。腹股溝沒有腫大淋巴結則不需做預防性淋巴結清除。但若合併皮膚侵犯性癌症,即使目前沒有可摸到的腫大淋巴結,亦可以考慮行淋巴結切片。當本身已有其它癌症的病人新診斷有陰莖陰囊處EMPD時,亦需注意癌症多發性轉移的問題。皮膚侵入性腺癌患者手術後建議做放射性治療。若有淋巴結轉移,則需行全身化學治療。 |
英文摘要 | Background:Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma that is associated with underlying malignancy. The common sites of EMPD are the vulva and anorectal and penoscrotal areas. There are limited reports on EMPD in the literature. The treatment plan for and prognosis of EMPD with associated malignancy remain to be clarified.Aim and Objectives:This study reported the clinical experience of penoscrotal EMPD with associated malignancy. Here, we have discussed the clinical experience at Taipei Veteran General Hospital and have provided a further treatment plan.Materials and Methods:From January 1985 to December 2011, 14 patients were diagnosed with penoscrotal EMPD with associated malignancy, and they received treatment at Taipei Veteran General Hospital. The clinical data were collected via a retrospective chart review. According to the different types of associated malignancies and the time of diagnosis, these patients were separated into 3 groups: EMPD with delayed malignancy, EMPD with invasive adenocarcinoma, and EMPD with pre-existing distant malignancy. The relationships among penoscrotal EMPD, associated malignancy, inguinal lymph node metastasis, and the outcome were analyzed.Results:Among patients with penoscrotal EMPD, 14 of 59 patients (23.7%) had invasive adenocarcinoma or an internal malignancy. In total, 11 internal malignancies (18.6%) and 4 invasive skin adenocarcinomas (6.8%) were diagnosed. The most common internal malignancy was prostate cancer (4/11, 36.4%). The average time to malignancy was 101 months (range: 60 - 211) after the EMPD diagnosis in the delayed malignancy group and 82.25 months (range: 28 - 120) before the EMPD diagnosis in the pre-existing distant malignancy group. Patients who had EMPD with delayed malignancy had a better outcome. Their 5-year survival rate was 100%. Three patients had inguinal lymph node metastasis and all of these patients had EMPD with invasive adenocarcinoma.Conclusion:Local wide excision was the standard treatment. Associated malignancy should be considered in patients with EMPD. The workup for genitourinary tract malignancy was recommended. Other evaluations were planned according to the patients' symptoms. Prophylactic lymph node dissection was not suggested in patients without palpable inguinal lymph nodes. EMPD with invasive adenocarcinoma frequently progressed to inguinal lymph nodes and distant organs. Lymph node biopsy was considered in this group even though there was no palpable node. Systemic survey was suggested for EMPD with pre-existing distant malignancy because multiple metastases were strongly suspected. Adjuvant radiotherapy was suggested for dermis or subcutaneous adenocarcinoma. Systemic chemotherapy was considered when inguinal lymph node metastasis was present. Long-term follow-up for more than 5 years is needed because local recurrence or secondary malignancy might occur after several years. |
本系統中英文摘要資訊取自各篇刊載內容。