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纵隔内胚窦瘤。7例报告并文献复习。

Endodermal sinus tumor of the mediastinum. A report of seven cases and review of the literature.

作者信息

Truong L D, Harris L, Mattioli C, Hawkins E, Lee A, Wheeler T, Lane M

出版信息

Cancer. 1986 Aug 1;58(3):730-9. doi: 10.1002/1097-0142(19860801)58:3<730::aid-cncr2820580321>3.0.co;2-o.

Abstract

Primary mediastinal endodermal sinus tumor is rare, and to date only 49 cases have been described in the English-language literature. Seven new cases are reported. Light microscopic examination showed characteristic features including papillary, reticular, tubular and solid growth patterns, complete or incomplete Schiller-Duval bodies and intracellular or extracellular periodic acid-Schiff-positive material. Immunohistochemical studies showed alpha-1-antitrypsin in seven, alpha-fetoprotein in seven, keratin in six, and carcinoembryonic antigen in four cases. The beta subunit of human chorionic gonadotropin, albumin, fibronectin, and transferrin were not found in any case. Electron microscopic studies performed in four cases showed intracellular and extracellular basement membrane-like material, multiple large multivesicular bodies, desmosomes and microvilli. Mediastinal endodermal sinus tumor can and must be differentiated from poorly differentiated adenocarcinoma metastatic or extending to the mediastinum because of the distinctly different prognoses and therapies. In spite of modern chemotherapy, the prognosis of mediastinal endodermal sinus tumor remains poor. The single most important prognostic indicator is whether the tumor mass can be completely excised before or after chemotherapy.

摘要

原发性纵隔内胚窦瘤罕见,迄今为止英文文献中仅报道过49例。本文报告了7例新病例。光镜检查显示其具有特征性表现,包括乳头状、网状、管状和实性生长模式、完整或不完整的席勒-杜瓦尔小体以及细胞内或细胞外的过碘酸希夫染色阳性物质。免疫组化研究显示,7例中有α1-抗胰蛋白酶阳性,7例中有甲胎蛋白阳性,6例中有角蛋白阳性,4例中有癌胚抗原阳性。所有病例均未发现人绒毛膜促性腺激素β亚基、白蛋白、纤连蛋白和转铁蛋白。4例进行了电镜检查,显示细胞内和细胞外有基底膜样物质、多个大的多囊泡体、桥粒和微绒毛。纵隔内胚窦瘤可以而且必须与转移至纵隔或蔓延至纵隔的低分化腺癌相鉴别,因为它们的预后和治疗方法明显不同。尽管有现代化疗手段,纵隔内胚窦瘤的预后仍然很差。最重要的预后指标是肿瘤肿块能否在化疗前或化疗后完全切除。

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