原发性阴茎阴囊Paget病22例临床分析

目的：探讨原发性阴茎阴囊Paget病的临床特点、治疗及预后。方法：回顾性分析2000年1月至2014年12月期间的22例原发性阴茎阴囊Paget病患者的临床、病理资料，随访其预后，采用Kaplan-Meier法进行生存分析。结果：22例患者的中位发病年龄64.5岁，中位病程40个月，中位病损长径4.75 cm。病理分期中，A1期患者12例，A2期患者6例，B期患者4例。12例患者阴囊阴茎均受累，仅侵犯阴囊者5例，仅侵犯阴茎者5例。患者的临床表现以红斑、瘙痒、溃烂、渗出及疼痛为主，均接受手术治疗，浸润性患者17例（77.3%），其中6例患者的手术切缘阳性，非浸润性患者的手术切缘无阳性发现。12例患者术后出现局部复发或转移，手术切缘阳性（P=0.015）和浸润性患者（P=0.010）更易出现局部复发或转移，腹股沟淋巴结为该病转移时最常受累的部位，是否有淋巴结转移（P=0.040）以及病灶的体积（P=0.027）与病程的长短存在相关性。总体的5年生存率和浸润性患者的5年生存率分别为33.7%和27.9%，手术切缘阳性的患者预后差。结论：原发性阴茎阴囊Paget病好发于老年人，具有病程长、易浸润、易复发和转移的特点，初步治疗应首选手术。延误诊断、手术切缘阳性及腹股沟淋巴结转移是其复发、转移的重要高危因素。术前组织病理检查、术中切缘冰冻切片病理检查及腹股沟淋巴结活检有助于规范临床诊疗。

Primary penoscrotal extra-mammary Paget’s disease: analysis of 22 cases

Objective:To investigate the clinical characteristics,treatment and prognosis of primary pe-noscrotal extra-mammary Paget’s disease (PSPD).Methods:The clinical and pathological data of 22 cases of primary PSPD were retrospectively reviewed.Survival rate of the overall patients,the invasive patients,and the patients with positive surgical margin or negative surgical margin were analyzed with Ka-plan-Meier survival curve method.Results:Among all the patients with primary PSPD,the median age of onset was 64.5 (39 -84)years,the median time of disease duration was 40 (2 -300)months,and the median long diameter of lesion was 4.75 (1 -10)cm.In the study,12 patients (54.5%)were in pathological stage A1,6 patients (27.3%)were in pathological stage A2,and 4 patients (18.2%) were in pathological stage B.Scrotum and penile of most patients (n =12,54.5%)were involved,5 patients (22.7%)were scrotum only,and 5 patients (22.7%)were penile only.Erythema (n =18, 81.8%),itchiness (n =16,72.7%),ulcerate (n =12,54.5%),exudation (n =11,50.0%),and pain (n =4,18.2%)were the major manifestations.All the patients with primary PSPD were treated with wide surgical excision.The rate of invasive patients was 77.3% (n =17).Of them,6 patients had positive surgical margin.The surgical margins of non-invasive patients were all negative.Twelve patients exhibited local recurrence or/and metastases,and the status of surgical margins (P =0.015)and the depth of invasion (P =0.010)were important risk factors.Inguinal lymph nodes were generally in-volved.The difference of the delay of diagnosis between metastasis and non-metastasis was significant (P =0.040).The 5-year survival rates of the overall and invasive patients were 33.7% and 27.9%, respectively.The patients with positive surgical margin had poor prognosis.Conclusion:Primary PSPD, with the characteristics of long duration,high invasive tendency and high incidence of local recurrence or metastases,generally occurs in the elderly.Surgery should be performed at first.The delay of diagnosis, positive surgical margins and the involvement of inguinal lymph node are important risk factors.Biopsy, frozen section and inguinal lymph node biopsy (ILNB)can standardize diagnosis and treatment.