乳房外Paget病19例临床与随访分析

Clinical Features and Follow up Analysis of 19 Patients with Extramammary Paget Disease

目的探讨乳房外Paget病的临床、病理特点及影响生存率的因素。方法回顾性分析2000—2010年间本院收治的19例乳房外Paget病患者的临床资料。结果19例中女4例，男15例，平均年龄（70．26±8．90）岁。确诊时病程1月-6．5年，平均43月。临床表现主要为红斑、糜烂及湿疹、皮炎样皮损。手术治疗13例，其中2例复发；光动力治疗5例；未治疗1例。随访期间死亡7例，5年生存率61．20％。单因素变量分析显示淋巴结转移、肿瘤细胞侵袭至真皮、伴发其他肿瘤或远处转移、CEA水平升高与患者生存率有关。结论乳房外Paget病早期诊断困难，复发率高，淋巴结转移、伴发肿瘤或远处转移者预后差，需长期随访。迁延不愈的“湿疹”患者应尽早行皮肤活检，免疫组化检查可提高早期诊断率并指导早期治疗。

Objective To evaluate the clinical and pathological features of extramammary Paget disease （EMPD） and investigate prognostic factors for survival. Methods The medical records of 19 patients with EMPD were retrospectively reviewed. Results The mean age at diagnosis was （70.26 ±8.90） years. The average time between onset and diagnosis was 43 month （ranged： 1 month - 6.5 years）. There were 4 females and 15 males. The most common symptom was muhifocat erythema, erosion, and eczematoid dermatitis. Of them, 13 patients were treated with surgery and 2 recurred, and 5 patients were treated with photodynamic therapy. One patient hadn＇t given treatment. Of the patients who were followed up, seven died. The 5-year overall survival rate for all patients was 61.20%. On univariate analysis,lymph node metastasis,tumor invasion level,secondary malignant or distant metastasis, and elevated serum carcinoembryonic antigen （CEA） levels were significant prognostic factors. Conelusions EMPD is difficult to be diagnosed at the initial time and the recurrence rate is high. Invasion level, secondary malignancy or distant metastasis, and lymph node metastasis are important prognostic factors in EMPD. Long time follow-up is necessary. Suspect patient should undergo skin and lymph node biopsy, lmmunohistochemistry is helpful to diagnose and treatment.