阴囊Paget病100例临床组织病理分析

Clinical ＆ histopathologieal analysis of 100 cases of scrotal Paget＇s disease

目的探讨100例阴囊Paget病的临床和组织病理改变。方法所有标本均来自1987—2004年复旦大学附属华山医院皮肤科，分析其临床资料、HE染色、特殊染色和免疫组化改变。结果肿瘤细胞有三种形态：经典型51例，黏液型或印戒细胞型11例；小细胞型18例，其他20例为混合细胞型。Paget细胞在表皮内扩散方式可分为稀疏型，旺炽型和经典型。肿瘤细胞可沿毛囊、汗腺管向下扩散，最后突破真表皮交界面和皮肤附属器基底膜而达真皮层。结论组织病理学特征是诊断阴囊Paget病最重要的线索，鉴别诊断包括浅表播散性恶性黑素瘤、皮肤淋巴瘤、Paget样原位鳞状细胞癌和继发性阴囊Paget病。

Objective To analyze the clinical and histopathological features of 100 cases of scrotal Paget＇s disease. Methods Specimens were collected from 100 outpatients with scrotal Paget＇s disease evaluated in the Department of Dermatology, Huashan Hospital, Fudan University during the period of 1987-2004. Clincal data as well as findings in the examination with H＆E staining, special staining and immunohisto-chemical staining were retrospectively assessed for these cases. Results Three types of tumor cells were observed in these specimens, including typical Paget cells （51 cases）, mucinous or signet-ring cells （ 11 cases）, small cells （18 cases）. Mixed types of tumor cells were seen in 20 cases. Paget cells spread in the epidermis in three patterns （sparsely, intensively and classically）. Tumor cells could extend down along the epithelium of follicles or sweat glands, ultimately broke through dermal-epidermal junction or the basal membrane of adnexa, and migrated into the dermis. Conclusions Histopathological examination is the most important clue to the diagnosis of scrotal Paget＇s disease. Histologically, it should be differentiated from superficial spreading malignant melanoma, skin lymphoma, pagetoid squamous cell carcinoma in situ and secondary scrotal Paget＇s disease.