Role of ploidy, chromosome 1p, and Schwann cells in the maturation of neuroblastoma
Role of ploidy, chromosome 1p, and Schwann cells in the maturation of neuroblastoma
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DOI：
10.1056/nejm199606063342304
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发表时间：
1996-06-06
影响因子：
158.5
通讯作者：
Ambros, PF
中科院分区：
文献类型：
--
作者：
Ambros, IM;Zellner, A;Ambros, PF
背景。神经母细胞瘤是一种异质性疾病，其表现范围从自发消退到致命扩散。有时肿瘤会自发分化为良性神经节神经瘤（成熟神经母细胞瘤）。预后通常与倍性、1 号染色体短臂缺失和 N-myc 癌基因扩增有关。成熟的神经母细胞瘤由神经元细胞和施万细胞组成。我们研究了成熟神经母细胞瘤中两种细胞类型的遗传组成，以确定遗传异常与成熟之间的关系。方法。我们通过原位杂交研究了 20 种正在成熟和成熟的神经母细胞瘤，对神经元细胞和雪旺细胞中的染色体进行计数并评估 1 号染色体短臂中可能的缺失。通过流式细胞术测量细胞的DNA含量。结果。神经母细胞和神经节细胞的染色体数量出现异常。原位杂交和流式细胞术证明 19 个肿瘤中有 18 个呈近三倍体，其余肿瘤呈五倍体。所有 20 个神经母细胞瘤中的雪旺细胞都含有正常数量的染色体。在研究的 18 个肿瘤中，两种类型的细胞均未出现 1 号染色体缺失。结论。成熟神经母细胞瘤中的雪旺细胞在遗传上与神经元细胞不同。雪旺细胞中染色体的正常数量以及神经母细胞和神经节细胞中的异常数量表明雪旺细胞是侵入神经母细胞瘤的正常细胞的反应性群体。神经母细胞瘤细胞的近三倍体和完整的 1 号染色体可能是自发类器官成熟的遗传先决条件，因为我们在自发成熟的神经母细胞瘤的神经元细胞中没有发现二倍体或 1 号染色体缺失。 (C) 1996 年，马萨诸塞州医学会。
Background. Neuroblastoma is a heterogeneous disease, with manifestations ranging from spontaneous regression to lethal spread. Sometimes the tumor spontaneously differentiates toward a benign ganglioneuroma (maturing neuroblastoma). The prognosis is frequently related to ploidy, deletions in the short arm of chromosome 1, and amplifications of the N-myc oncogene. Maturing neuroblastomas consist of both neuronal cells and Schwann cells. We investigated the genetic composition of both cell types in maturing neuroblastomas, to determine the relation between genetic abnormalities and maturation.Methods. We studied 20 maturing and mature neuroblastomas by in situ hybridization to count the chromosomes and evaluate possible deletions in the short arm of chromosome 1 in neuronal and Schwann cells. The DNA content of the cells was measured by flow cytometry.Results. Neuroblastic and ganglionic cells showed aberrations in the number of chromosomes. In situ hybridization and flow cytometry demonstrated near-triploidy in 18 of 19 tumors and pentaploidy in the remaining tumor. The Schwann cells in all 20 neuroblastomas contained normal numbers of chromosomes. In 18 tumors studied, there were no chromosome 1 deletions in either type of cell.Conclusions. The Schwann cells in maturing neuroblastomas differ genetically from the neuronal cells. The normal number of chromosomes in Schwann cells and the abnormal number in neuroblastic and ganglionic cells suggest that Schwann cells are a reactive population of normal cells that invade the neuroblastoma. Near-triploidy of neuroblastoma cells and intact chromosome 1 are presumably genetic prerequisites for spontaneous organoid maturation, because we found no diploidy or chromosome 1 deletions in the neuronal cells of spontaneously maturing neuroblastormas. (C) 1996, Massachusetts Medical Society.