NEURON-SPECIFIC ENOLASE IN CHILDHOOD TUMORS

儿童期实体瘤的诊断和治疗通常 由于缺乏明显的特征而很难。 这是 尤其是尤文氏肉瘤，神经母细胞瘤，原始软组织 肉瘤和（偶尔）淋巴瘤。 我们已经评估了 特异性神经酶，神经元特异性烯醇化酶（NSE）， 各种儿童实体肿瘤的石蜡包埋切片， 包括以前未被识别的神经肿瘤变体， 用NSE抗血清进行免疫细胞化学染色。 我们发现， 所有的神经肿瘤都有这种抗体 与非神经性 肿瘤，除了一个罕见的分化横纹肌肉瘤的例子，被发现。 我们的结论是，NSE是一个可靠的，容易检测的标志物，即使在 儿童神经源性肿瘤 此外，我们还定义了 一种新描述的胸壁“圆细胞”肿瘤的神经组织发生 类似于尤文氏肉瘤，也就是所谓的Askin瘤， 一种周围神经上皮瘤。 最后，我们最近证实， 所谓的周围神经上皮瘤的独特特征， NSE阳性，但显示混合神经和许旺形态学 特色

The diagnosis, and thus therapy, of solid tumors of childhood is often difficult due to lack of distinguishing characteristics. This is especially true of Ewing's sarcoma, neuroblastoma, primitive soft tissue sarcomas, and (occasionally) lymphoma. We have evaluated the presence of a specific neural enzyme, neurone-specific enolase (NSE), in paraffin-embedded sections of a diverse group of solid childhood tumors, including previously unrecognized variants of neural tumors, employing immunocytochemistry with antisera to NSE. We find uniform reactivity of all neural tumors with this antibody. No cross-reactivity with non-neural tumors, save a rare example of differentiated rhabdomyosarcoma, was found. We conclude that NSE is a reliable, readily detected marker in even primitive childhood tumors of neural origin. Also, we have defined the neural histogenesis of a newly described, "round cell" tumor of chest wall resembling Ewing's sarcoma, the so-called Askin tumor, which in reality is a form of peripheral neuroepithelioma. Finally, we have recently confirmed the unique character of so-called peripheral neuroepithelioma, which is NSE-positive but which displays hybrid neural and Schwannian morphologic characteristics.