Cosgarea I, Zaremba A, Hillen U
Dermatological Sciences, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, Großbritannien.
Klinik für Dermatologie, Universitätsklinikum Essen, Essen, Deutschland.
Hautarzt. 2019 Sep;70(9):670-676. doi: 10.1007/s00105-019-4459-8.
Extramammary Paget's disease (EPD) is a rare, slowly growing, cutaneous adenocarcinoma with an incidence of 0.1-2.4 per 1,000,000 inhabitants. Histologically, EPD is characterized by the presence of epidermal Paget's cells, similarly to mammary Paget's disease. The EPD is typically divided into primary EPD (type I) and secondary EPD (type II associated with colorectal carcinoma and type III associated with urogenital carcinoma). From a clinical point of view, EPD is unspecific commonly mimicking chronic inflammatory skin disorders. This unspecific clinical picture can impede and delay the diagnosis of EPD. The treatment of choice for local EPD is the micrographically controlled excision. The extent of the infiltration of adnexal structures should be histologically determined prior to topical therapies, such as imiquimod and superficial ablative therapy. The complete excision of the tumor can be challenging due to ill-defined borders. In the metastatic stage the EPD has a poor prognosis. Controlled clinical trials for systemic treatment are still lacking.
乳房外佩吉特病（EPD）是一种罕见的、生长缓慢的皮肤腺癌，发病率为每100万居民中有0.1 - 2.4例。从组织学上看，EPD的特征是存在表皮佩吉特细胞，这与乳腺佩吉特病相似。EPD通常分为原发性EPD（I型）和继发性EPD（II型与结直肠癌相关，III型与泌尿生殖系统癌相关）。从临床角度来看，EPD缺乏特异性，通常类似慢性炎症性皮肤病。这种缺乏特异性的临床表现会妨碍和延迟EPD的诊断。局部EPD的首选治疗方法是在显微镜控制下切除。在进行局部治疗（如咪喹莫特和浅表消融治疗）之前，应通过组织学确定附属结构的浸润范围。由于边界不明确，完整切除肿瘤可能具有挑战性。在转移阶段，EPD的预后较差。目前仍缺乏系统性治疗的对照临床试验。
