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肝脏原发性混合性生殖细胞肿瘤,伴肉瘤样成分。

Primary mixed germ cell tumor of the liver with sarcomatous components.

机构信息

Third Department of Intervention Radiotherapy, Eastern Hepatobiliary Surgery Hospital, Second Military Medical University, 225 Changhai Road, Shanghai 200438, China.

出版信息

World J Gastroenterol. 2010 Feb 7;16(5):652-6. doi: 10.3748/wjg.v16.i5.652.

Abstract

Germ cell tumor (GCT) of the liver is extremely rare. Here, we describe a case of hepatic mixed GCT with significant sarcomatous components and elevated serum alpha-fetoprotein (AFP) in a 34-year-old man. Histopathologically, the tumor was composed of two GCTs components: yolk sac tumor and immature teratoma. The predominant components of immature teratoma consisted of several types of tissue that represented different germinal layers (endoderm, mesoderm and ectoderm) and showed varying degrees of differentiation with significant sarcomatous components. The yolk sac component showed positivity for AFP and cytokeratin (AE1/AE3). The immature teratoma components showed positivity for varying differentiation markers. Interphase cytogenetic analysis revealed that the yolk sac tumor and immature teratoma were positive for i(12p) and 12p over-representation. In particular, the rhabdomyoblastic components also showed typical i(12p) and 12p overrepresentation. This suggested that sarcomatous components may be associated with dedifferentiation or malignant transformation of certain mesenchymal components within teratoma.

摘要

肝生殖细胞肿瘤 (GCT) 极为罕见。在此,我们描述了 1 例 34 岁男性肝混合性 GCT,其肉瘤成分显著,并伴有血清甲胎蛋白 (AFP) 升高。组织病理学上,肿瘤由两种 GCT 成分组成:卵黄囊瘤和未成熟畸胎瘤。未成熟畸胎瘤的主要成分由几种类组织组成,代表不同的胚层(内胚层、中胚层和外胚层),并显示出不同程度的分化,伴有显著的肉瘤成分。卵黄囊成分对 AFP 和细胞角蛋白 (AE1/AE3) 呈阳性。未成熟畸胎瘤成分对不同的分化标志物呈阳性。间期细胞遗传学分析显示,卵黄囊瘤和未成熟畸胎瘤均存在 i(12p) 和 12p 过度表达。特别是横纹肌样成分也表现出典型的 i(12p) 和 12p 过度表达。这表明肉瘤成分可能与畸胎瘤内某些间充质成分的去分化或恶性转化有关。

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