乳腺外Paget’s病的临床特点及其预后分析

Clinical Features and Prognosis of Extramammary Paget’s Disease

目的探讨乳腺外Paget's病(EMPD)的临床特点、复发的危险因素及影响预后的因素。方法回顾性分析2009~2019年就诊于我院的41例确诊为乳腺外Paget's病患者的资料,随访生存状况,分析发病年龄、性别、病程、病灶面积、手术切缘、肿瘤细胞有无浸润、病理切缘是否阳性、有无淋巴结转移以及有无附属器官肿瘤与其预后的关系。结果41例患者中男性34例(82.93%),女性7例(17.07%),发病年龄50~85岁。38例(92.68%)接受了手术切除治疗,其中浸润性患者17例(44.74%)。38例手术患者中8例术后出现复发,其中4例死亡。发病年龄、性别、病程、手术切缘对患者生存状况无明显影响(P>0.05),病灶面积较大、病理切缘阳性、肿瘤细胞浸润性生长、伴有淋巴结转移均可降低患者无瘤生存期(P<0.05)。结论EMPD常好发于老年男性,具有病程长、易复发、易浸润的特点,因其皮损表现,早期常被误诊为湿疹、皮炎等。治疗首选手术切除,皮损面积大、切缘阳性、淋巴结转移、肿瘤呈侵袭性生长、远处转移等均为影响肿瘤复发及患者预后的重要高危因素。

Objective To explore the clinical characteristics of extramammary Paget's disease(EMPD),risk factors for recurrence,and factors affecting prognosis.Methods The data of 41 patients diagnosed with extramammary Paget's disease diagnosed in our hospital from 2009 to 2019 were retrospectively analyzed,and the survival status was followed up.Whether the pathological margin is positive,whether there is lymph node metastasis,and whether there is a relationship between tumors of accessory organs and their prognosis.Results Among the 41 patients,34 were male(82.93%)and 7 were female(17.07%).The age of onset was 50 to 85 years.38 patients(92.68%)underwent surgical resection,of which 17 were invasive patients(44.74%).Recurrence occurred in 8 of the 38 surgical patients,4 of whom died.Age of onset,gender,course of disease,and surgical margin have no significant effect on patient survival(P>0.05).Larger lesions,positive pathologic margins,invasive growth of tumor cells,and lymph node metastasis can all reduce tumor-free survival period(P<0.05).Conclusion EMPD often occurs in elderly men.It has the characteristics of long course,recurrence,and infiltration.Because of its skin lesions,it is often misdiagnosed as eczema and dermatitis in the early stage.Surgical resection is the first choice for treatment.Large skin lesions,positive margins,lymph node metastases,aggressive tumor growth,and distant metastases are important high-risk factors that affect tumor recurrence and patient prognosis.