Jedrych Jaroslaw, Leffell David, McNiff Jennifer M
Department of Pathology, Yale School of Medicine, New Haven, CT, USA.
J Cutan Pathol. 2012 Mar;39(3):317-23. doi: 10.1111/j.1600-0560.2012.01876.x.
Desmoplastic trichoepithelioma (DTE) is a benign follicular tumor occurring most commonly within facial skin of young and middle-aged women, morphologically characterized by a superficial dermal proliferation of basaloid cells growing in narrow strands embedded in a desmoplastic stroma associated with small keratinizing cysts. DTE must be distinguished from other benign epithelial proliferations such as syringoma, microcystic adnexal carcinoma and infiltrating basal cell carcinoma. Among morphological features useful in that distinction, perineural involvement is considered a feature indicative of malignancy. We present a series of seven DTEs with otherwise typical presentation and morphology, nevertheless showing epithelium present in the perineural spaces of adjacent small dermal nerves. Patients ranged in age from 14 to 66 years (mean 44 years). All seven tumors were restricted to dermis, showed strands of basaloid epithelium in desmoplastic stroma and contained CK20-positive cells. Additionally, five of five examined tumors displayed diffuse expression of p75 neurotrophin receptor. Five patients were followed up clinically (follow-up time range: 2 months-4 years). No tumor recurrence was observed in any of these patients. We postulate that perineural involvement is an unusual feature of DTE that should not be equated with malignancy or lead to unnecessary over-treatment.
促结缔组织增生性毛发上皮瘤(DTE)是一种良性毛囊肿瘤,最常见于中青年女性的面部皮肤,其形态学特征为基底样细胞在真皮浅层呈窄束状增生,嵌入促结缔组织增生性基质中,并伴有小的角化囊肿。DTE必须与其他良性上皮增生性病变相鉴别,如汗腺腺瘤、微囊性附属器癌和浸润性基底细胞癌。在有助于鉴别诊断的形态学特征中,神经周围侵犯被认为是恶性肿瘤的一个特征。我们报告了7例DTE病例,其临床表现和形态学表现均较为典型,但在相邻小皮神经的神经周围间隙中可见上皮组织。患者年龄在14至66岁之间(平均44岁)。所有7个肿瘤均局限于真皮层,在促结缔组织增生性基质中可见基底样上皮束,并含有细胞角蛋白20(CK20)阳性细胞。此外,在5个接受检查的肿瘤中,有5个显示p75神经营养因子受体弥漫性表达。对5例患者进行了临床随访(随访时间为2个月至4年)。这些患者均未观察到肿瘤复发。我们推测,神经周围侵犯是DTE的一种不常见特征,不应将其等同于恶性肿瘤,也不应导致不必要的过度治疗。