NEURON-SPECIFIC ENOLASE IN CHILDHOOD TUMORS

儿童实体瘤的诊断和治疗是 通常由于缺乏显著特征而难以实现。 这 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。 我们有 评估了一种特殊的神经酶的存在，神经元， 特异性烯醇化酶（NSE），在石蜡包埋的切片中， 儿童实体肿瘤的不同群体，包括以前 神经肿瘤的未识别变体，采用 用NSE抗血清进行免疫细胞化学染色。 我们找到制服 所有神经肿瘤与这种抗体的反应性。 没有十字架- 与非神经肿瘤的反应，除了一个罕见的例子， 分化型横纹肌肉瘤。我们的结论是， NSE是一种可靠的，容易检测的标记物， 儿童神经源性肿瘤 此外，我们还定义了 神经组织发生的一个新的描述，“圆细胞”肿瘤， 胸壁类似尤文肉瘤，即所谓的Askin 肿瘤，这实际上是一种形式的外周神经上皮瘤。 最后，我们最近证实了这种独特的性质- 称为外周神经上皮瘤，NSE阳性， 它表现出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 neuronal 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.