- 期刊
Perianal Paget's Disease: Presentation of Twelve Cases and a Literature Review
肛門周圍之乳房外柏哲德氏症之系列案例分析及文獻回顧
摘要
目的：肛門周圍柏哲德氏症，是一種罕見皮膚的惡性腫瘤，約佔乳房外柏哲德氏症20%，目前有提出論文討論的病例數200例左右，手術治療為主要方法，但復發率在統計上仍偏高。因此，我們收集彰化基督教醫院案例進行分析，並回顧相關文獻。方法：本研究回溯本院1995年至2014年間，診斷為「肛門周圍柏哲德氏症」之12位患者。我們記錄並分析其年紀、性別、臨床表現、確診時間、病理報告、術前診斷、手術方法與復發及死亡與否，另搜尋1990-2014可得論文共針對213例患者治療文獻做回顧及討論。結果：12位患者中平均年齡為70.8歲（65-76歲）。其中三位（25%）是女性。其中十一位接受手術切除。平均追蹤時間為92.1個月。只有一位患者死於肝癌，其餘患者皆存活。接受手術切除的患者中只有一位有復發情形，此病人拒絕再次接受廣泛性切除，因此安排放射治療（32 Gy/16 fractions），追蹤四年後，無復發情況發生。結論：手術切除是目前認為最主要的治療方法，但非手術的治療方式，對於不希望手術或不適合手術，且病理上為非侵犯性的肛門周圍之乳房外柏哲德氏症之病人，或許是可行的方法，因病例數不多，未來仍需更多的研究來證明此論點。
並列摘要
Purpose. Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma. To date, ~200 cases have been reported, representing ~20% of all extramammary Paget's disease cases. The standard treatment is wide local excision, although PPD has a high recurrence rate. Here, we review our management for PPD and review the currently recommended treatment approaches. Methods. In this study, we reviewed 12 patients diagnosed with PPD between 1995 and 2014. Clinical data including age, sex, symptoms, symptom duration, histopathology, treatment modality, recurrence, follow-up duration, and survival outcome were evaluated. We also reviewed the published literature from 1990 to 2014 to identify the treatment strategies used in 213 cases of PPD. Results. Twelve patients with in situ disease with a median age of 70.8 (65-76) years, including 3 female patients (25%), were diagnosed with PPD. Eleven patients underwent surgery. The mean follow-up time was 92.1 (36-200) months. One patient died from liver cancer and bone metastasis, and the remaining patients are alive; 10 are disease-free and 1 experienced postsurgical local recurrence. This patient refused extensive radical excision, so radiotherapy (32 Gy in 16 fractions) was administered and the patient remains disease-free after 4 years. Conclusion. Surgery is the mainstay treatment for PPD. Nonsurgical modalities could be considered an alternative to surgery for those with noninvasive PPD who refuse radical surgery or who are medically unfit for surgery. Further research and follow-up are needed to compare the effectiveness of surgical and nonsurgical therapeutic modalities for PPD.