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Penosrcrotal Extrammarry Paget's Disease with Invasive Ductal Carcinoma of the Breast-Report of a Case and Review of Literature

乳房外陰莖陰囊Paget氏病合併男性侵犯性乳腺管癌─病例報告及文獻回顧

摘要


背景:陰莖陰囊之乳房外Paget氏病(EMPD)出現於男性乳癌非常罕見。由於EMPD這種疾病之非特異性病徵及少見,延遲診斷是可能的,須特別留意。目的及目標:本文報告一乳房外陰莖陰囊Paget氏病出現於有男性乳癌病史之極少見病例,並進行文獻回顧以釐清EMPD之治療策略。材料及方法:一位71歲男性病人在診斷出陰莖陰囊Paget氏病之前4年,曾經進行乳癌切除手術。這次陰莖陰囊之病灶呈現硬幣大小,紅且癢的現象。腹股溝摸不到淋巴病變,診斷出EMPD之後進行大範圍切除及股溝皮瓣重建手術。結果:術後恢復過程順利,局部再給予imiquimod藥膏。追蹤二年間病人未出現局部復發,體內癌症,遠位轉移或股溝淋巴病變。結論:乳房外Paget氏病可能出現於男性乳癌病患,當病人出現長期皮膚濕疹或潰瘍,且一般內科治療無效時,即應高度懷疑此病。手術切除是EMPD治療的基石,然而這種皮膚腫瘤的真正侵犯邊緣可能不清楚,不明確,須進行組織學確認才行。

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並列摘要


Background:Penoscrotal Extramammary Paget's Disease (EMPD) occur in a male patient with breast cancer is extremely rare, which is seldom discussed in the literature. Due to the nonspecific characters and rarity of EMPD, delayed diagnosis is possible, which should be paid with special attention.Aims and Objectives:To present an extremely rare case of penoscrotal extramammary Paget's disease occurred in a male patient with breast cancer history and to review literature to clarify the strategy in managing EMPD.Materials and Methods:A 71-year-old man who experienced right breast cancer and underwent mastectomy 4 years prior to this occurrence of his penoscrotal lesion was treated. The lesion appeared to be coin-sized, mildly erythematous and pruritic, over left penoscrotal area without inguinal lymphadenopathy. Under the diagnosis of extramammary Paget's disease, a wide excision and reconstruction with inguinal transposition flaps were done.Results:The postoperative course was smooth. Topical Imiquimod cream was prescribed after surgery. At 2-year follow-up, no local recurrence, internal malignancy, distant metastasis, inguinal lymphadenopathy was noted.Conclusion:EMPA can occur in male patient with history of breast cancer. A high index of suspicion should always be the rule with patients presenting with long standing eczematous and/or ulcerative lesion non responsive to medical treatment. Surgery is the foundation of treatment for Extramammary Paget's Disease. However, the true margin of this skin tumor may be inconspicuous and not delineated, which should be confirmed on histological examination during surgery.

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