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多发性遗传性毛发上皮瘤背景下发生的恶性毛母质瘤(“恶性毛发上皮瘤”)

Malignant hair matrix tumor ("malignant trichoepithelioma") arising in the setting of multiple hereditary trichoepithelioma.

作者信息

Hunt S J, Abell E

机构信息

Department of Dermatology, University of Pittsburgh, Pennsylvania.

出版信息

Am J Dermatopathol. 1991 Jun;13(3):275-81. doi: 10.1097/00000372-199106000-00010.

DOI:10.1097/00000372-199106000-00010
PMID:1867358
Abstract

An unusual adnexal tumor on the scalp of a 59-year-old woman with hereditary trichoepithelioma is reported. After the initial removal, the tumor recurred twice during a 4-year period. Mohs micrographic surgery resulted in full excision of the lesion. Two years later there was no evidence of recurrence. Histologic examination of the tumor showed it to merge with adjacent areas of classic trichoepithelioma. The tumor showed cytologic atypia, mitotic figures, necrosis, deep infiltration, and features of pilar differentiation including clear cells, reminiscent of cells of the follicular outer root sheath, and focal shadow-cell formation. The differential diagnosis of this malignant follicular neoplasm included pilomatrix carcinoma and basal-cell carcinoma with matrical differentiation. The unique clinicopathologic features, however, raised consideration of a "malignant trichoepithelioma."

摘要

报告了一名患有遗传性毛发上皮瘤的59岁女性头皮上的罕见附件肿瘤。初次切除后,肿瘤在4年期间复发了两次。莫氏显微外科手术完全切除了病变。两年后没有复发迹象。肿瘤的组织学检查显示它与经典毛发上皮瘤的相邻区域融合。肿瘤表现出细胞异型性、有丝分裂象、坏死、深层浸润以及毛囊分化特征,包括透明细胞,让人联想到毛囊外根鞘细胞,还有局灶性影细胞形成。这种恶性毛囊肿瘤的鉴别诊断包括毛母质癌和具有母质分化的基底细胞癌。然而,独特的临床病理特征引发了对“恶性毛发上皮瘤”的考虑。

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Malignant hair matrix tumor ("malignant trichoepithelioma") arising in the setting of multiple hereditary trichoepithelioma.多发性遗传性毛发上皮瘤背景下发生的恶性毛母质瘤(“恶性毛发上皮瘤”)
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引用本文的文献

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Trichoblastic carcinoma: a systematic review of an aggressive follicular neoplasm with an emphasis on Mohs micrographic surgery in management.毛囊母质癌:一种侵袭性毛囊肿瘤的系统综述,重点关注莫氏显微外科手术在其治疗中的应用。
Arch Dermatol Res. 2025 May 25;317(1):783. doi: 10.1007/s00403-025-04275-y.
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Metastatic trichoblastic carcinoma in the setting of trichoblastomatosis and multiple facial trichoepitheliomas.毛囊母细胞瘤病和多发性面部毛发上皮瘤背景下的转移性毛母质癌
JAAD Case Rep. 2021 Sep 1;16:127-129. doi: 10.1016/j.jdcr.2021.08.024. eCollection 2021 Oct.
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A case of multiple familial trichoepitheliomas responding to treatment with the Hedgehog signaling pathway inhibitor vismodegib.
一例多发性家族性毛发上皮瘤对 Hedgehog 信号通路抑制剂维莫德吉治疗有反应。
Virchows Arch. 2018 Aug;473(2):241-246. doi: 10.1007/s00428-018-2397-y. Epub 2018 Jun 22.
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Twelve Years' Observation of Multiple Familial Trichoepithelioma with Squamous Carcinoma.多发性家族性毛发上皮瘤伴鳞状细胞癌的十二年观察
Indian J Dermatol. 2016 May-Jun;61(3):348. doi: 10.4103/0019-5154.182464.
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Skin adnexal neoplasms--part 1: an approach to tumours of the pilosebaceous unit.皮肤附属器肿瘤——第1部分:皮脂腺单位肿瘤的诊治方法
J Clin Pathol. 2007 Feb;60(2):129-44. doi: 10.1136/jcp.2006.040337. Epub 2006 Aug 1.