Introduction

We reviewed the medical and surgical management and long-term outcomes for patients diagnosed with penoscrotal extramammary Pagets disease (EMPD) within an eUROGEN centre.

Patients and Methods

Retrospective review of cases from an institutional database with biopsy proven penoscrotal EMPD.

Results

A total of 10 patients were identified with penoscrotal EMPD over a 10-year period. Two patients had a previous history of gastrointestinal and urogenital cancers (20%) and no synchronous or metachronous cancers were identified. Eight patients with non-invasive EMPD (80%) underwent wide local excision of the affected skin, with at least a 5mm macroscopic resection margin and in selected cases simultaneous multiple mapping biopsies around the lesion were performed. Residual disease was present at the margins in seven patients (87.5%), of which three required further surgical excision or adjuvant topical immunotherapy (42.8%). Recurrence after complete excision was 12.5% and was successfully treated with topical imiquimod immunotherapy and CO₂ laser therapy. Two patients (20%) had invasive carcinoma and metastatic disease at diagnosis.

Conclusion

Reported recurrence rates of non-invasive penoscrotal EMPD are high and residual disease is present in most cases requiring either close clinical surveillance or adjuvant treatment. We propose an algorithm in the management of this rare disease.

中文翻译：

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管理阴茎阴囊外佩吉特病的多学科方法——一项 eUROGEN 研究

介绍

我们审查了 eUROGEN 中心内诊断为阴囊外佩吉特病 (EMPD) 的患者的内科和外科管理以及长期结果。

患者和方法

对来自机构数据库的病例进行回顾性审查，活检证明阴囊 EMPD。

结果

在 10 年的时间里，共有 10 名患者被确定为阴囊 EMPD。两名患者既往有胃肠道和泌尿生殖系统癌症病史 (20%)，未发现同步或异时性癌症。8 名非侵入性 EMPD 患者 (80%) 接受了受影响皮肤的广泛局部切除，宏观切除边缘至少为 5 毫米，并且在选定的情况下，同时在病变周围进行了多次定位活检。7 名患者 (87.5%) 的边缘存在残留病灶，其中 3 名需要进一步手术切除或辅助局部免疫治疗 (42.8%)。完全切除后的复发率为 12.5%，并通过局部咪喹莫特免疫疗法和 CO ₂成功治疗激光治疗。两名患者 (20%) 在诊断时患有浸润性癌和转移性疾病。

结论

据报道，非侵入性阴囊 EMPD 的复发率很高，并且在大多数需要密切临床监测或辅助治疗的病例中存在残留疾病。我们提出了一种管理这种罕见疾病的算法。