增生性外毛根鞘瘤31例临床及组织病理分析

Clinicopathological analysis on 31 cases of proliferating trichilemmal cyst

目的:探讨增生性外毛根鞘瘤(PTC)患者的临床、组织病理学特征及鉴别诊断。方法:对31例确诊PTC患者的临床和组织病理资料进行回顾性分析并复习相关文献。结果:31例PTC患者中,男16例,女15例,年龄12～89岁,平均54.45岁,病程2周～40年,平均75.88个月。皮损面部最多,19例,占61.29%。本组31例患者中,只有1例临床诊断PTC,准确率仅3.23%,误诊率96.77%。PTC组织病理特征包括真皮内多发性肿瘤结节/囊肿、非浸润性栅栏状边界、透明基膜带、外毛根鞘样骤然角化。结论:PTC临床容易误诊,诊断主要依靠组织病理特征,应与基底细胞癌、鳞状细胞癌、恶性PTC及外毛根鞘癌等鉴别。

Objective： To study the clinicopathological features and differentiation of proliferating trichilemmal cyst （PTC）. Methods： The clinical and pathological data on 31 cases of histopathologically confirmed PTC were retrospectively analyzed. And, the related literature was reviewed. Results： In total 31 patients with PTC were reviewed, including 16 males and 15 females, from 12 to 89 years of age, with an average of 54.45 years. The disease course ranged from 2 weeks to 40 years, with an average of 75.88 months. The skin lesions mostly located on the face of the patients, which were found in 19 cases out of 31 cases of PTC （61.29%）. Only one case was correctly diagnosed in clinic. The accuracy of diagnosis was only 3.23% while the misdiagnosis rate reached 96.77%. The histopathology showed several features of PTC, including multiple tumor nodules and cysts in the dermis, non-invasive peripheral palisade arrangement of the tumor cells, transparent basement membrane zone and sudden trichilemmal keratosis. Conclusions： PTC is easily to be misdiagnosed in clinic. Correct diagnosis is mainly based on the histopathological features. Differential diagnosis of PTC in- cludes basal cell carcinoma, squamous cell carcinoma, malignant proliferating trichilemmal tumor and trichilemmal carcinoma.