摘要目的 探讨外毛根鞘癌的临床和组织病理学特征.方法 收集13例外毛根鞘癌患者的临床及组织病理资料.结果 13例患者中男9例,女4例,年龄34～87岁,平均70岁.临床表现为突出皮肤表面的肿块.镜下肿瘤组织呈实体状、叶状、小梁状,以伴有外毛根鞘角化的上皮细胞增生为特征,主要由不典型透明细胞构成.13例均行过碘酸雪夫染色,肿瘤透明细胞均阳性.6例做了免疫组化染色,均表现为瘤细胞表达高分子角蛋白、上皮膜抗原,不表达癌胚抗原、S-100、细胞角蛋白8、上皮抗原(Ber-Ep4).9例随访4个月至5年,无复发和转移.结论 外毛根鞘癌是一种低度恶性的皮肤附属器肿瘤,缺乏可辨认的临床特征,需与皮肤其他恶性透明细胞瘤鉴别.

abstractsObjective To investigate the clinical and histological characteristics of trichilemmal carcinoma (TLC). Methods A clinicopathological analysis of 13 cases of TLC was carried out. Results There were 9 males and 4 females among the 13 patients with TLC who were aged from 34 to 87 years (mean: 70 years). Clinically, the tumor presented as an exophytic mass; histologically, it was characterized by the proliferation of epithelial cells and keratinization of outer root sheath. Cytologically atypical clear cells predominated in the tumor tissue. Microscopy revealed different growth patterns of tumor cells, which included solid growth pattern, tobular pattern and trabecular pattern. Periodic acid Schiff (PAS) stain demonstrated clear cells in all the tumor tissues from the 13 patients. Immunohistochemistry was performed in tissue samples from 6 patients, and showed that these samples were positive to high molecular weight cytokeratin (CK-HMW) and epithelial membrane antigen (EMA), but negative to carcinomebrynic antigen (CEA), S-100, cytokeratin 8 (CK8)and epithelial antigen(Ber-Ep4). Follow-up over 4 months to 5 years revealed neither recurrence nor metastasis in 9 cases.Conclusions TLC is a low-grade malignancy of skin adnexal tumor without distinctive clinical features, and should be differentiated from other malignant clear cell tumors of the skin.