视网膜母细胞瘤小鼠模型中的中脑原始神经外胚层肿瘤

Primitive neuroectodermal tumor of the midbrain in a murine model of retinoblastoma.

作者信息

Marcus D M, Carpenter J L, O'Brien J M, Kivela T, Brauner E, Tarkkanen A, Virtanen I, Albert D M

机构信息

David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.

出版信息

Invest Ophthalmol Vis Sci. 1991 Feb;32(2):293-301.

PMID:1993580

Abstract

The first heritable model of retinoblastoma was established by retina-specific expression of simian virus 40 T-antigen (SV40 T-ag) in transgenic mice. Bilateral, multifocal ocular tumors were observed in 100% of transgene-bearing mice. Central nervous system neoplasms occurred at a lower rate (27%) and represented the murine counterpart of human trilateral retinoblastoma. The authors characterized the transgenic brain tumors and found them to be primitive neuroectodermal tumors (PNET) of the midbrain. Murine brain tumors do not involve the pineal gland and most closely resemble undifferentiated suprasellar or parasellar tumors occasionally observed in human trilateral retinoblastoma. The murine malignancies arose from the subependymal cells of the cerebral aqueduct. Immunohistochemical and ultrastructural examination revealed that the transgenic brain tumors were undifferentiated and lacked all antigens associated with normal murine neuronal, glial, and ependymal cells.

摘要

通过在转基因小鼠中视网膜特异性表达猿猴病毒40 T抗原（SV40 T-ag），建立了视网膜母细胞瘤的首个可遗传模型。在100%携带转基因的小鼠中观察到双侧、多灶性眼肿瘤。中枢神经系统肿瘤发生率较低（27%），代表人类三侧性视网膜母细胞瘤的小鼠对应物。作者对转基因脑肿瘤进行了特征描述，发现它们是中脑的原始神经外胚层肿瘤（PNET）。小鼠脑肿瘤不涉及松果体，最类似于人类三侧性视网膜母细胞瘤中偶尔观察到的未分化鞍上或鞍旁肿瘤。小鼠恶性肿瘤起源于中脑导水管的室管膜下细胞。免疫组织化学和超微结构检查显示，转基因脑肿瘤未分化，缺乏与正常小鼠神经元、神经胶质和室管膜细胞相关的所有抗原。