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促结缔组织增生性毛发上皮瘤

Desmoplastic Trichoepithelioma

作者信息

Taylor Alexandra, Rice Ashley S.

机构信息

CUSOM/Sampson Regional Medical Center

PMID:39383281
Abstract

Desmoplastic trichoepithelioma (DTE) is an uncommon benign adnexal tumor originating from the hair follicle, first described by Zeligman in 1960 as a "solitary trichoepithelioma." The lesion is classified into 3 subgroups: multiple familial trichoepithelioma, solitary nonhereditary trichoepithelioma, and desmoplastic trichoepithelioma. In 1977, MacDonald et al referred to DTE as a "sclerosing epithelial hamartoma," and Brownstein and Shapiro later coined the term "desmoplastic trichoepithelioma," which remains in use today. Typically presenting as an asymptomatic annular, papular nodule with a "thread-like elevated border," DTE is usually <2 cm in diameter and commonly occurs in young to middle-aged women, particularly in cosmetically sensitive areas such as the cheeks, forehead, and chin. Histopathologically, DTE is characterized by nests and cords of basaloid cells within a dense, fibrous stroma, occasionally containing small cystic spaces filled with keratinous material. Peripheral palisading of cells is observed; significant mitotic activity is generally absent. These features distinguish DTE from other skin neoplasms like basal cell carcinoma (BCC) and microcystic adnexal carcinoma, which exhibit more aggressive histologic features. Clinically, DTE manifests as a slow-growing, skin-colored facial plaque or nodule with a depressed center, developing very slowly before stabilizing. Despite its benign nature and low risk of malignant transformation, DTE tends to grow if untreated and has a propensity for recurrence due to its poorly circumscribed histologic growth pattern. Familial cases have been reported, suggesting a potential genetic predisposition. Often an incidental finding during routine skin cancer examinations, DTE can frequently be diagnosed clinically by experienced dermatologists. The preferred treatment is surgical excision, with Mohs micrographic surgery recommended to ensure clear margins, especially in cosmetically challenging areas like the face. DTE is a unique tumor due to its benign nature, distinct histopathological features, and superficial invasion. Accurate diagnosis relies on careful clinical and histopathological evaluation to distinguish it from more aggressive neoplasms.

摘要

促结缔组织增生性毛发上皮瘤(DTE)是一种罕见的源于毛囊的良性附属器肿瘤,1960年由泽利格曼首次描述为“孤立性毛发上皮瘤”。该病变分为3个亚组:多发性家族性毛发上皮瘤、孤立性非遗传性毛发上皮瘤和促结缔组织增生性毛发上皮瘤。1977年,麦克唐纳等人将DTE称为“硬化性上皮错构瘤”,布朗斯坦和夏皮罗后来创造了“促结缔组织增生性毛发上皮瘤”这个术语,至今仍在使用。DTE通常表现为无症状的环状、丘疹样结节,有“线状隆起边缘”,直径通常<2 cm,常见于年轻至中年女性,尤其是在面部、前额和下巴等对美容敏感的区域。组织病理学上,DTE的特征是在致密的纤维基质内有基底样细胞巢和索,偶尔含有充满角质物质的小囊腔。观察到细胞的外周栅栏状排列;一般无明显的有丝分裂活性。这些特征将DTE与其他皮肤肿瘤如基底细胞癌(BCC)和微囊性附属器癌区分开来,后者表现出更具侵袭性的组织学特征。临床上,DTE表现为生长缓慢、肤色的面部斑块或结节,中央凹陷,在稳定之前发展非常缓慢。尽管其本质为良性且恶性转化风险低,但如果不治疗,DTE往往会生长,并且由于其组织学生长模式边界不清,有复发倾向。有家族性病例报告,提示可能存在遗传易感性。DTE通常是在常规皮肤癌检查中偶然发现的,经验丰富的皮肤科医生通常可以通过临床诊断。首选治疗方法是手术切除,建议采用莫氏显微描记手术以确保切缘清晰,尤其是在面部等对美容有挑战性的区域。DTE是一种独特的肿瘤,因其良性本质、独特的组织病理学特征和浅表侵袭性。准确的诊断依赖于仔细的临床和组织病理学评估,以将其与更具侵袭性的肿瘤区分开来。

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Desmoplastic Trichoepithelioma促结缔组织增生性毛发上皮瘤
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Desmoplastic trichoepithelioma, infiltrative/morpheaform BCC, and microcystic adnexal carcinoma: differentiation by immunohistochemistry and determining the need for Mohs micrographic surgery.促纤维增生性毛发上皮瘤、浸润性/硬斑病样基底细胞癌和微囊性附属器癌:通过免疫组织化学进行鉴别及确定是否需要莫氏显微外科手术
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