乳腺外Paget病41例临床特征及预后分析

Analysis on the clinical features and prognosis in 41 cases of extramammary Paget′s disease

目的 探讨乳腺外Paget病(EMPD)的常见临床特征,包括流行病学、临床表现、病理特点、手术治疗并分析影响预后的因素。方法 回顾性分析2015年1月至2021年1月由郑州大学第一附属医院收治的41例乳腺外Paget病患者的流行病学特征,临床表现、组织病理、手术治疗及预后情况。分析临床参数与预后的关系,并进行统计学分析。结果 41例EMPD患者男女比例3.1∶1,中位发病年龄为65岁,60岁以上患者有32例(78%)。37例(90.2%)患者病变累及于生殖器区域,临床表现主要为湿疹样红斑、糜烂并伴有瘙痒。所有患者均采用手术治疗,扩大切除范围1~3 cm。应用χ^(2)检验得出,扩切范围<2 cm组与扩切范围≥2 cm组、有术中快速冰冻组与无术中快速冰冻组、有淋巴结转移组和无淋巴结转移组、有真皮浸润组和无真皮浸润组的复发率比较,差异有统计学意义(P<0.05)。患者不同年龄、发病至就诊时间、病变范围间复发率比较,差异无统计学意义(P>0.05)。结论 EMPD多发生于中老年男性的生殖器部位,临床表现不特异。局部扩大切除手术是其主要的手术方式,扩切范围2 cm以上,并行术中快速冰冻检查切缘,可降低术后复发率。肿瘤伴真皮浸润、淋巴结转移时复发率升高。

Objective To investigate the common clinical features of extramammary Paget′s disease(EMPD),including epidemiological features,clinical manifestations,pathological characteristics and surgical treatment methods,and to analyze the factors affecting prognosis.Methods The epidemiological features,clinical manifestations,histopathology,surgical treatment methods and prognosis of 41 patients with EMPD admitted to the First Affiliated Hospital of Zhengzhou University from January 2015 to January 2021 were retrospectively analyzed.The correlation between clinical parameters and prognosis was analyzed and the statistical analysis was performed.Results The 41 patients with EMPD had a male-to-female ratio of 3.1∶1,with a median age at disease onset of 65 years and 32 patients(78%)over 60 years of age.37 patients(90.2%)had lesions involving the genital area,with eczema-like erythema,erosions and pruritus as major clinical manifestations.All patients were treated with surgery,and the excision range was expanded by 1-3 cm.The chi-square test showed that there were statistically significant differences in relapse rates between the groups with expanded excision range<2 cm and≥2 cm,between the groups with and without intraoperative rapid freezing,between the groups with and without lymph node metastasis,and between the groups with and without dermal invasion(P<0.05).There were no significant differences in relapse rates between groups with different patient ages,time from disease onset to consultation,and extents of lesion(P>0.05).Conclusion EMPD occurs mostly in the genital area of middle-aged and aged men with nonspecific clinical manifestations.Expanded local excision is the main surgical procedure for this disease,and the postoperative relapse rate can be reduced when the excision range is expanded by≥2 cm,together with intraoperative rapid freezing examination of the cut edge.The relapse rate is higher when the tumor is accompanied by dermal invasion and lymph node metastasis.