外毛根鞘癌5例临床病理特征及文献复习  

Tricholemmal carcinoma: clinicopathological features and review of literature

作　　者：陈荣明[1] 蚁国铮[2] 

机构地区：[1]长丰县人民医院病理科,安徽长丰231100 [2]安徽省立医院病理科,合肥230001

出　　处：《诊断病理学杂志》2008年第6期462-466,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨外毛根鞘癌（TLC）的临床病理特征、诊断及鉴别诊断。方法对5例外毛根鞘癌进行临床病理学、组织化学观察和分析并复习相关文献。结果5例均为女性,年龄20-61岁,平均39岁。肿瘤直径1.2-6 cm,平均3.6 cm。临床表现为皮下实性结节或突出于皮肤表面的蕈样肿块,发生于头皮、面部及颈部皮肤。镜下肿瘤组织呈分叶状、梁索状,主要由不典型透明细胞构成。其中2例做了PAS染色,透明细胞（＋）;3例随访4-11年无复发和转移。结论TLC是低度恶性肿瘤,具有局部侵袭性、罕见复发、基本上不发生转移的特点。诊断主要靠分叶状结构和外毛根鞘分化的证据,特别是肿瘤主要成分为不典型透明细胞、外毛根鞘型角化者。鉴别诊断包括恶性增生性外毛根鞘瘤、皮肤鳞状细胞癌、毛母质癌、皮脂腺癌等。手术完整切除,预后好。Objective To explore the clinicopathological features, diagnosis and differential diagnosis of tricholemmal carcinoma （TLC）. Methods 5 cases of TLC were observed and analyzed by means of clinicopathologic data analysis, hematoxylin-eosin and histochemical staining. In addition, the related literature was reviewed. Results All of 5 cases were female, aged 20-61 years （mean age 39 years）. The tumor diameter varied from 1.2 cm to 6 cm （mean 3.6 cm）. Clinically, the tumors presented as either solid subcutaneous nodules or exophytic fungoid mass showing predilection for the scalp and the skin of face and neck. Histologically, each TLC consisted of a lobular （nest-like） or trabecular infrltrative epithelial proliferation, in which cytologicallyatypical clear cells predominate. PAS stain was performed in 2 cases, with PAS-positivity in the clear cells. Follow-up in 3 cases revealed neither recurrence nor metastasis over a period of 4-11 years. Conclusion TLC is a low-grade malignancy of the skin with an indolent clinical course, which appears to be locally aggressive, rarely recurrent and virtually unable to metastasize. The pathological diagnosis of TLC depends mainly on the intradermal multilobular architecture and the proofs of external hair root sheath differentiation, particularly a predominant number of atypical clear cells and abrupt tricholemmal-type keratinization. The differential diagnosis of TLC might include malignant proliferating trichilemmal tumor, squamous cell carcinoma of the skin, pilomatrical carcinoma and sebaceous carcinoma. Complete surgical resection is recommended in therapy.

关 键 词：外毛根鞘癌 临床病理学 鉴别诊断 

分 类 号：R739.5[医药卫生—肿瘤]