Zhu Yao, Ye Ding W, Chen Zhong W, Zhang Shi L, Qin Xiao J
Department of Urology, Fudan University Cancer Hospital, Shanghai, China.
BJU Int. 2007 Dec;100(6):1282-7. doi: 10.1111/j.1464-410X.2007.07188.x. Epub 2007 Sep 10.
To analyse the clinicopathological characteristics of penoscrotal extramammary Paget's disease (EMPD) and to discuss the outcomes after frozen section-guided wide local excision.
From 1990 to 2005, at our institution, 38 patients with penoscrotal EMPD received wide local excision with intraoperative frozen-section analysis. Their medical records were reviewed for patient demographics, lesion characteristics, surgical margin status, and clinical outcome.
No patients had EMPD secondary to a non-cutaneous malignancy; 23 patients had intraepithelial EMPD, 12 had invasive EMPD and three had EMPD with underlying adnexal adenocarcinoma. The median (range) largest diameter of the lesion was 6 (1-20) cm. Of 38 patients, 12 (32%) had positive frozen-section results and had extended surgical excision until a negative margin was obtained. Additional margin examination of the gross specimen and final examination of the frozen sections showed that five patients had false-negative results. There was a microscopic positive margin in 15 of 38 (40%) patients when a conventional 2 cm clinical tumour-free border was maintained. Skin erythematous patches were significantly correlated with the spread of disease (P = 0.03). After a median (range) follow-up of 33.5 (3-140) months, six of 38 (16%) patients had recurrent disease, of whom only two had recurrent skin lesions, while four had systemic progression.
Frozen section-guided wide local excision gave an acceptable recurrence rate in the treatment of penoscrotal EMPD. Primary EMPD with dermal invasion should be actively monitored for possible aggressive behaviour.
分析阴茎阴囊部乳房外佩吉特病（EMPD）的临床病理特征，并探讨冰冻切片引导下广泛局部切除术后的结局。
1990年至2005年，在本机构，38例阴茎阴囊部EMPD患者接受了术中冰冻切片分析的广泛局部切除。回顾他们的病历以了解患者人口统计学、病变特征、手术切缘状态和临床结局。
无患者的EMPD继发于非皮肤恶性肿瘤；23例患者为上皮内EMPD，12例为浸润性EMPD，3例为伴有附属器腺癌的EMPD。病变的最大直径中位数（范围）为6（1 - 20）cm。38例患者中，12例（32%）冰冻切片结果为阳性，接受了扩大手术切除直至切缘阴性。对大体标本进行额外的切缘检查以及对冰冻切片进行最终检查显示，5例患者有假阴性结果。当保持传统的2 cm临床无肿瘤边界时，38例（40%）患者中有15例存在显微镜下阳性切缘。皮肤红斑与疾病扩散显著相关（P = 0.03）。中位（范围）随访33.5（3 - 140）个月后，38例患者中有6例（16%）出现疾病复发，其中只有2例有皮肤病变复发，而4例有全身进展。
冰冻切片引导下广泛局部切除在阴茎阴囊部EMPD的治疗中复发率可接受。对于伴有真皮浸润的原发性EMPD，应积极监测其可能的侵袭性行为。
