乳头状汗腺瘤9例临床病理分析

Hidradenoma papilliferum:a clinicopathologic analysis of 9 cases

目的研究乳头状汗腺瘤的临床病理特点和免疫学表型。方法收集三峡大学第一临床医学院病理科1995～2009年存档和诊断的9例乳头状汗腺瘤的临床及病理资料并对切除的标本进行免疫组织化学染色,抗体包括ER、PR、CK(AE1/AE3)、CK5/6、CK7、CK20、CK(Low MW)、CK34βE12、EMA、p63、actin、CD10、S-100、Calponin、GCDFP-15和vimentin。结果9例患者平均发病年龄48岁,男女比例1∶8,主要发生于外阴及肛周。临床表现为单发的直径0.5～1.2 cm大小的半球形、孤立的、界限清楚的皮下结节;组织病理可见肿瘤由大小不一的腺泡、腺管和复杂折叠的乳头状结构组成,乳头的轴心为纤维血管。腺腔和乳头结构内衬单层柱状上皮细胞,可见顶浆分泌;在柱状细胞外周见梭形或立方形的肌上皮细胞,肌上皮与内层柱状细胞形成双层排列。免疫组化检测显示:9例肿瘤的内层柱状细胞均表达ER、PR、CK(AE1/AE3)、CK(Low MW)、CK7和EMA,7例表达CK34βE12,所有肿瘤的肌上皮细胞均表达p63、actin、CD10、S-100和Calponin,7例肿瘤的内层柱状细胞与肌上皮同时表达CK5/6,所有的肿瘤细胞均不表达CK20、GCDFP-15和vimentin,但纤维间质均表达vimentin。结论乳头状汗腺瘤是一种少见的皮肤良性肿瘤,临床表现多样,极易误诊。确诊依赖于组织病理检查。完整切除肿物后不复发。

Purpose To study the clinicopathologic characteristics and immunophenotype of hidradenoma papilliferum,a rare benign neoplasm of the skin.Methods The clinical and histopathologic data were reviewed and studied in nine cases of hidradenoma papilliferum,which were collected from the Department of Pathology,the First College of Clinical Medical Science,China Three Gorges University from 1995 to 2009.All specimens were stained by immunohistochemical method with a panel of antibodies including ER,PR,CK（AE1/AE3）,CK5/6,CK7,CK20,CK（low MW）,CK34βE12,EMA,p63,actin,CD10,S-100,Calponin,GCDFP-15 and vimentin.Results The typical hidradenoma papilliferum occured almost exclusively in the skin of the anogenital region.The mean age was 48 years.The ratio of male to female was 1 to 8.Vulva and anal areas were the main locations and the lesion of only one male case occurred on the armpit.Clinically,the lesions of the skin presented a solitary,firm,freely mobile and well demarcated subcutaneous nodule.The tumors were 0.5 cm to 1.0 cm in diameter.Microscopically,a well-circumscribed,cystic lesion in the mid dermis was surrounded by a fibrous capsule with no connection to the overlying epidermis.Variously shaped cystic,tubular and papillary structures were present in the tumor.The papillary structure had a central fibrovascular core.The lumina of those structures were usually lined with a double layer of cells consisting of a luminal layer of tall columnar cells that showed active decapitation secretion similar to that seen in secretory cells of apocrine glands,and an outer one or two layers of cuboidal,flattened myoepithelial cells.Immunohistochemistry showed the epithelial cells of all the tumors were positive for ER,PR,CK（AE1/AE3）,CK（low MW）,CK7 and EMA.The epithelial cells of 7 cases also strongly expressed CK34βE12.The myoepithelial cells of all cases were positive for p63,actin,CD10,S-100 and Calponin.CK5/6 were distributed in epithelial cells and myoepithelial cells in 7 cases.All tumors were negative for CK20,GCDFP-15 and vimentin.But the stromal elements expressed vimentin.Conclusion Hidradenoma papilliferum is a rare benign tumor of the skin.It shows a diversified clinical characteristics and is often misdiagnosed.Diagnosis is based on histopathology.No recurrence is observed after a complete surgical excision.