乳房外Paget病5例临床分析

Clinical analysis of 5 cases with extramammary Paget′s disease

目的：提高对乳房外Paget病的诊治水平。方法：分析2000年～2007年我院收治的5例乳腺外Paget病的临床表现及诊治方法。结果：所有手术标本均行病理学检查确诊，1例切口脂肪液化，经Ⅱ期缝合痊愈，余Ⅰ期愈合。5例患者均得到随访，平均随访时间（4．0±0．6）年。所有患者局部无复发，1例出现腹膜后广泛浸润，死亡；1例死于他病，余存活。结论：临床应提高对本病的警惕，可疑时及时行皮肤活检，免疫组化对于明确诊断有很大价值。术中标明方位的多点快速冰冻切片确定切缘阴性是提高病人生存率的关键，治疗效果较好。

Objective：To improve the level of diagnosis and treatment of extramammary Paget＇s disease. Methods ： Five cases of extramammary Paget＇s disease were reviewed with regard to the clinical manifestation, diagnosis and management from 2000 to 2007 in our hospital. Results ： The 5 cases were confirmed pathologically to be extramammary Paget＇s disease, among whom 1 case complicated with the skin lesion spreading to the scrotum and penile required second closure of the skin before healing but the remaining 4 got primary healing. The follow-up in average 4.5 years suggested that no local tumor recurrence except for 1 cases died from retroperitoneal lymph node metastases and another 1 died of unknown disease. Conclusion ： The suspected lesions like eczema in perineal region should undergo skin biopsy for early diagnosis and immunohistochemistry can be effective approach to the confirmation of extramammary Paget＇s disease. The intraoperative rapid frozen biopsy from multiple regions for defining the incisal edge may result in a higher rate of survival.