乳头状汗腺瘤10例临床病理分析

Hidradenoma papilliferum:a clinicopathologic analysis of 10 cases

目的探讨乳头状汗腺瘤的临床病理特征、诊断与鉴别诊断。方法对10例乳头状汗腺瘤的临床病理特征和免疫组化表型分析。结果本组女性9例,男性1例,年龄31~65岁,平均44岁,9例位于外阴和肛周,1例位于肩颈部;临床表现为单发丘疹或界限清楚的皮下结节,直径0.5~2 cm;组织学上见肿瘤由大小不一的腺管和相互吻合的乳头状结构组成,被覆双层上皮,内层为高柱状的上皮细胞,可见顶浆分泌,外层为梭形或矮立方的肌上皮细胞。免疫组化显示：所有肿瘤的内层柱状细胞均ER、PR、CK和EMA（＋）,9例CK34βE12和CK7（＋）,3例AR和GCDFP-15（＋）,1例CK20（＋）,所有肿瘤肌上皮细胞均p63和S-100（＋）,7例肿瘤双层上皮CK5/6同时（＋）,Ki-67增值指数从1%~60%不等。结论乳头状汗腺瘤是一种少见的发生于肛周生殖器的皮肤良性肿瘤,个别肿瘤可发生癌变,因临床表现多变,极易误诊,通过组织活检可确诊。肿瘤完整切除后预后良好。

Objective To study the clinicopathologic features,diagnosis and differential diagnosis of hidradenoma papilliferum.Methods Ten cases of hidradenoma papilliferum were observed for the morphological features and immunophenotype.Results Among the 10 cases studied,1 was male and 9 were female,aged 44 years（range from 31 to 65 years）.Nine pathological bases were located the vulva and anal areas,and the lesion of only one male case occurred on the shoulder of the patient.Clinically,the lesions of the skin presented a solitary,firm,freely mobile and well demarcated subcutaneous nodule.The tumors were 0.5 cm to 2.0 cm in diameter.Histopathological examination using the biopsy speciment revealed that the major components of the neoplasm consisted of tubular formations and papillary proliferation characteristic of neoplastic cells.The tubular structures consisted of two layers of pleomorphic cells：columnar cells with decapitation secretion in the luminal layer and the cuboidal cells in the basal layer.Immunohistochemistry showed the epithelial cells of all the tumors were positive for ER,PR,CK（AE1/AE3） and EMA.9 cases of epithelial cells in all patients also expressed CK34βE12 and CK7,3 cases expressed AR and GCDFP-15,and 1 case expressed CK20.The myoepithelial cells of all cases were positive for p63 and S-100.CK5/6 were distributed in epithelial cells and myoepithelial cells in 7 cases.Ki-67 proliferation index ranged from 1% to 60%.Conclusions Hidradenoma papilliferum is a rare benign tumor of the vulva.Some cases can change from benign tumor to cancer.It shows a diversified clinical characteristics and is often misdiagnosed.Diagnosis is based on the histopathology.Resecting tumor completely indicates favorable prognosis.