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目的:分析腋窝乳房外Paget病(EMPD)的临床及病理特点。方法:报告1例左腋窝EMPD患者,并回顾分析国内报道的11例类似病例。结果:12例腋窝EMPD患者中,男9例,女3例,男女比例为3∶1,年龄89~47岁,平均发病年龄68.75岁;均为单侧发病,左、右两侧各6例,左∶右=1∶1,病程2~30年,皮损初为界限清楚的红斑,病程长者可出现糜烂、溃疡。有2例分别并发大汗腺癌及肺癌。组织病理显示:表皮角化过度,表皮内基底层及棘层可见明显增大的异形肿瘤细胞,肿瘤细胞胞质丰富、透亮,胞核呈类圆形,空泡状,核仁增大、清晰,近表皮处可见异形细胞,基底层完整,真皮浅层可见炎性细胞浸润。免疫组化染色显示:癌胚抗原(CEA)强阳性,上皮膜抗原(EMA)和角蛋白(CK)-7,癌胚抗原增殖指数(Ki-67)>85%阳性。CK-20不表达。所有病例均手术切除,随访未发现复发。结论:腋窝EMPD是EMPD中的少见类型,首选手术切除,预后好,复发率低。
Objective: To analyze the clinical and pathological features of axillary extramammary Paget’s disease (EMPD). Methods: One case of left axillary EMPD was reported and 11 similar cases reported in China were retrospectively analyzed. Results: 12 cases of axillary EMPD patients, 9 males and 3 females, male to female ratio of 3: 1, aged 89 to 47 years, mean age 68.75 years of age; all unilateral disease, left and right sides of the 6 cases , Left: right = 1: 1, duration of 2 to 30 years, early lesions for the well-defined erythema, the elderly may appear erosions, ulcers. There are 2 cases of concurrent sweating adenocarcinoma and lung cancer. Histopathology showed that there were hyperkeratosis of epidermis, abnormally-shaped tumor cells with obviously enlarged basal and spine layers in the epidermis. The cytoplasm of the tumor cells were rich and translucent, and the nuclei were round, vacuole, nucleolus enlarged and clear , Near the epidermis visible abnormal cells, the basal layer integrity, dermal shallow inflammatory cell infiltration. Immunohistochemical staining showed that carcinoembryonic antigen (CEA) strongly positive, epithelial membrane antigen (EMA) and keratin (CK) -7, carcinoembryonic antigen proliferation index (Ki-67)> 85% positive. CK-20 is not expressed. All cases were surgically removed, no recurrence was found. Conclusion: Axillary EMPD is a rare type of EMPD, the preferred surgical resection, the prognosis is good, the recurrence rate is low.