Ota S, Crabbe D C, Tran T N, Triche T J, Shimada H
Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, CA 90027.
Cancer. 1993 May 1;71(9):2862-72. doi: 10.1002/1097-0142(19930501)71:9<2862::aid-cncr2820710930>3.0.co;2-d.
Malignant rhabdoid tumor (MRT), originally described as a rare renal sarcoma in childhood, has been known to express phenotypic diversity. In this study, unique characteristics of the MRT cells were investigated by using established cell lines.
Immunocytochemical, ultrastructural, cytogenetic, and molecular (by polymerase chain reaction, PCR) analyses were done for two MRT cell lines, one of renal and one of extrarenal origin, before and after differentiation-induction with either 12-O-tetradecanoyl phorbol-13-acetate (TPA) or transretinoic acid (RA).
The proliferating cells in the original tumor tissues as well as in the established cell lines demonstrated neural, epithelial, and mesenchymal markers morphologically. Both cell lines had karyotypic abnormalities including chromosome 22q11.2. The cell line from the extrarenal MRT, Tm87-16, demonstrated distinct morphologic changes with neuroblastic differentiation and produced numerous neuritic processes after treatment with either TPA or RA. The cell line from the renal MRT, STM91-01, suggested schwannian differentiation but did not change morphologically after chemical induction. Both cell lines expressed c-myc, but did not express N-myc, MyoD1, tyrosine hydroxylase, or neural cell adhesion molecule (N-CAM). With PCR and immunocytochemical study, a high level of chromogranin expression was detected by the cells of Tm87-16 only after TPA induced differentiation.
MRT cells demonstrated diverse phenotype of neuro-ecto-mesenchymal differentiation. The results of this study suggest that MRT may be derived from a primitive pluripotential cell, such as neural crest or equivalent. MRT, therefore, might be categorized as one of the subsets of primitive neuroectodermal tumor.
恶性横纹肌样瘤（MRT）最初被描述为儿童期罕见的肾肉瘤，已知其具有表型多样性。在本研究中，利用已建立的细胞系对MRT细胞的独特特征进行了研究。
对两个MRT细胞系（一个源于肾脏，一个源于肾外）在用12 - O - 十四烷酰佛波醇 - 13 - 乙酸酯（TPA）或全反式维甲酸（RA）诱导分化前后进行了免疫细胞化学、超微结构、细胞遗传学和分子（通过聚合酶链反应，PCR）分析。
原始肿瘤组织以及已建立的细胞系中的增殖细胞在形态上表现出神经、上皮和间充质标志物。两个细胞系均有染色体核型异常，包括22q11.2染色体。来自肾外MRT的细胞系Tm87 - 16在用TPA或RA处理后表现出明显的形态学变化，伴有神经母细胞分化并产生大量神经突起。来自肾MRT的细胞系STM91 - 01提示有雪旺氏细胞分化，但化学诱导后形态未改变。两个细胞系均表达c - myc，但不表达N - myc、MyoD1、酪氨酸羟化酶或神经细胞黏附分子（N - CAM）。通过PCR和免疫细胞化学研究，仅在TPA诱导分化后，Tm87 - 16细胞检测到高水平的嗜铬粒蛋白表达。
MRT细胞表现出神经 - 外胚层 - 间充质分化的多样表型。本研究结果提示MRT可能起源于原始多能细胞，如神经嵴或其等同物。因此，MRT可能被归类为原始神经外胚层肿瘤的一个亚群。
