Invasive Vulvular Extramammary Paget's Disease with HER-2/neu Overexpression── A Case Report

外陰部侵襲性乳房外柏杰氏病併Her-2/neu過度表現－病例報告

謝綺瀅(Chi-Ying Hsieh) ； 張閔翔(Min-Hsiang Chang) ； 陳右昇(Yo-Shen Chen) ； 游彥辰(Yen-Chen Yu) ； 阮廷倫(Tyng-Luen Roan) ； 張克中(Ke-Chung Chang) ； 林佐武(Tsuo-Wu Lin)

《臺灣整形外科醫學會雜誌》 26卷3期 (2017/09) Pp. 338-346

摘要

Background: Extramammary Paget’s disease (EMPD) is an uncommon intraepithelial malignancy, and few cases develop into invasive or metastatic adenocarcinoma. Similar to breast cancer, Her-2/neu overexpression in EMPD has been also considered as a prognostic factor for high recurrence rates and poor prognosis. Aim and Objectives: We use immunohistochemistry study to identify Her-2/neu and we would like to raise attentions to the correlation between overexpressed Her-2/neu and the severity of the disease. Furthermore, with more studies, Her-2/neu positive patients may benefit from targeted therapy to Her-2/neu. Materials and Methods: We report a case of invasive vulvular EMPD in a 60-year-old woman, who had eczematoid plaques with multiple nodules in the perineum. She underwent wide excision with right lymph node dissection and split-thickness skin graft for reconstruction. She recovered well postoperatively. Results: A microscopic examination revealed widespread EMPD and multifocal invasive adenocarcinomas. An immunohistochemical study found that the tumor cells were positive for Her-2/neu protein, which might explain the EMPD aggressiveness in this patient. Conclusions: First, we would like to review this rare but increasing encountered disease. More researches could be devoted to the overexpressed Her-2/neu and the severity of the EMPD disease. Furthermore, anti-Her-2/neu drugs may be an option of adjuvant therapy.

關鍵字

extramammary paget’s disease ； EMPD ； HER-2 ； vulvular

並列摘要

背景：乳房外柏杰氏病為罕見之表皮內惡性腫瘤，少數病人會發展成侵襲性癌症。部分案例會類似乳癌過度表現Her-2/neu蛋白質，這類案例可能會有較高的復發率與較差的存活率。目的及目標：我們利用免疫染色方法找出Her-2/neu蛋白質，希望藉由這個案例拋磚引玉提高大家對這個基因表現的注意，而能有更多研究來找出有Her-2/neu這類案例與疾病嚴重程度的關聯。進一步，希望能提供以Her-2/neu基因為標靶治療的可能性。材料及方法：我們報告一位六十歲女性有外陰部侵襲性乳房外柏杰氏病，其初期表現為外陰部類濕疹性斑塊與多發腫瘤，病人接受外陰切除與淋巴採驗後發現為乳房外柏杰氏病合併多發腺癌。手術治療主要採取外陰廣泛切除與分層植皮手術，術後復原情況良好，三年追蹤並未復發。結果：免疫染色發現腫瘤細胞過度表現Her-2/neu蛋白，可能解釋該病人病況為何較嚴重。結論： 一來希望藉此病例提高對此一罕見疾病之認知，二來希望更多研究能進一步探討腫瘤細胞過度表現Her-2/neu蛋白與疾病的嚴重程度，未來進而研究考慮標靶治療的成效。