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乳房外Paget病33例临床及组织病理分析

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目的 分析乳房外Paget病(EMPD)的临床及组织病理特征.方法 回顾性分析33例EMPD患者的临床和组织病理学资料.结果 33例患者中,男26例,女7例,平均发病年龄(70.46±8.79)岁.21例(63.6%)累及阴囊,7例(21.2%)累及外阴,3例(9.1%)累及阴茎,2例(6.1%)累及阴囊、阴茎.组织病理学检查显示原位型EMPD患者22例(66.7%),浸润真皮者10例(30.3%),伴有腹股沟淋巴结转移者9例(27.3%),合并汗腺癌4例(12.1%).除1例患者放弃治疗外,32例(96.7%)患者接受局部扩大切除术,并且切缘均阴性;术后随访发生腹股沟淋巴结转移9例(27.3%),均接受放射治疗.统计学数据显示乳房外Paget病发生淋巴结转移与患者年龄、性别、发病到确诊病程之间无显著相关性(P>0.05),与真皮浸润有显著相关性(P<0.05).结论 EMPD好发于外阴或阴囊部位,且其发生淋巴结转移与真皮浸润有相关性.建议侵袭性EMPD患者术后辅助局部放疗,可能会降低淋巴结转移风险.
Clinical and histopathological analysis of 33 patients with extramammary Paget's disease
Objective To analyze the clinical and pathological characteristics of extramammary Paget's disease(EMPD).Methods The clinical and histopathological data of 33 patients with EMPD were retrospectively analyzed.Results Among the 33 patients,26 were male and 7 were female,with an average age of disease onset of(70.46±8.79)years old.The analysis of lesion locations showed that 21 cases(63.6%)were confined to the scrotum,7 cases(21.2%)to the vulva,3 cases(9.1%)to the penis,and 2 cases(6.1%)affected both the scrotum and penis.The histopathological examination revealed that 22 cases(66.7%)presented with in situ EMPD,10 cases(30.3%)with dermal infiltration,9 cases(27.3%)with inguinal lymph node metastasis,and 4 cases(12.1%)with sweat gland cancer.The 32 patients(96.7%)underwent local enlarged resection,and all margins were negative.In the postoperative follow-up,9 patients(27.3%)developed inguinal lymph node metastasis and all received radiotherapy.Statistical data indicated that there was no significant association between lymph node metastasis of EMPD and the patient's age,gender,or the duration from onset to diagnosis(P>0.05).However,a significant correlation was observed with dermal infiltration(P<0.05).Conclusion The vulva or scrotum is the most common site for EMPD.Lymph node metastasis in EMPD is associated with dermal infiltration.suggesting that postoperative adjuvant local radiotherapy in patients with aggressive EMPD may reduce the risk of lymph node metastasis.It is proposed that postoperative adjuvant local radiotherapy for patients with aggressive EMPD may decrease the risk of lymph node metastasis.

Extramammary Paget's diseasePathological tissueTreatmentPrognosis

柴圆圆、严翘、董正邦、王飞

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215000 苏州,苏州大学附属儿童医院

东南大学附属中大医院

乳房外Paget病 病理组织 治疗 预后

2024

实用皮肤病学杂志
中国人民解放军北京军区总医院

实用皮肤病学杂志

CSTPCD
影响因子:0.637
ISSN:1674-1293
年,卷(期):2024.17(6)