阴囊Paget病26例临床分析

目的 探讨阴囊Paget病的临床特点、诊断及治疗方法.方法 对26例阴囊Paget病患者的临床资料进行回顾性分析,本组按Ray分期:A1期16例,A2期7例,B期3例.全部患者先行患部活检,后手术治疗.切除肉眼所见病变区域,切缘距病灶边缘2 cm,深度达深筋膜,18例行快速冰冻切片检查,肿瘤细胞累及切缘者3例(16.67%)予以扩大切除范围.1例术后病理证实累及切缘,立即再予手术治疗,3例经病理证实腹股沟淋巴结转移者行单侧或双侧腹股沟淋巴结清扫.结果 26例患者病理检查均见Paget病的典型特征;15例肿瘤局限于表皮,11例侵犯真皮;9例淋巴结肿大者,病理证实为淋巴结转移3例,淋巴结炎6例;26例均获随访,随访时间1～7年,平均5年.5例(19.23%)术后局部复发,再次手术切除后痊愈.结论 对疑为阴囊Paget病者应尽早行组织学检查,确诊后病灶根治性切除是首选的治疗方法,对局部复发者可行二次手术,预后良好.

Abstract：

Objective To investigate the clinical features, diagnosis and therapeutics of extramammary Paget′s disease. Methods A total of 26 cases of scrotal Paget′s disease were included. According to Ray staging, 16, 7 and 3 cases had A1, A2 and B stages of Peget′s diseases respectively. All cases underwent surgical resection of their diseased sites after a biopsy. The excision margin was 2 cm apart from the edge of lesion. And resection depth reached deep fascia. During operation, frozen sections of excised tissue specimens in 18 cases were pathologically examined. And the excision margins were involved by tumor cell in 3 cases(16.67%). Thus extended excision was performed immediately. Postoperatively, excision margin involvement was confirmed in 1 case and phaseⅡoperation performed. Three cases with inguinal lymph node metastasis underwent unilateral or bilateral inguinal lymph node dissection. Results Paget′s disease was confirmed in all 26 cases. Tumor was localized in epidemis in 15 cases and skin invasion found in 11 cases. The lymph node was tumescent in 9 cases. Among them, 3 had pathologically confirmed metastasis and 6 was diagnosed with lymphadenitis. All cases received a mean follow-up period of 5 years (range: 1-7 years). Five locally recurrent cases healed after a second operation. Conclusion Chronic skin lesions of the scrotum should be biopsied as early as possible if extramammary Paget′s disease is suspected. Radical resection of diseased skin is its preferred therapy. A second operation may be performed for recurrence. The prognosis of extramammary Paget′s disease is excellent after radical surgery.