PERIPHERAL NEUROEPITHELIOMA - A TUMOR DISTINCT FROM NEUROBLASTOMA

多年来，周围神经上皮瘤(PN)一直被低估， 因为它在大多数情况下与尤因肉瘤的组织学相似，或者 偶尔会发生横纹肌肉瘤(RMS)。最近，我们能够证明 胸肺小圆细胞瘤的神经外胚层性质 PN的变种--Askin区域(Askin Tumor)，使用免疫过氧化物酶 检测神经元特异性烯醇化酶(NSE)的存在和 电子显微镜(EM)。在对小圆细胞肿瘤的更广泛的研究中 童年时期，我们还发现NSE只存在于神经外胚层 肿瘤而不是尤因肉瘤或淋巴瘤。然而，横纹肌肉瘤 (均方根)对NSE也呈非特异性阳性，因此，对于 RMS与PN、肌肉标记物和EM的鉴别是另一项诊断 工具。 在这里，我们评估了组织学，免疫细胞化学，细胞化学和EM 15例PN的特点及其与临床表现的关系。 所有肿瘤均位于肾上腺外，发生在不同的软组织和骨质中。 节后部位。所有患者的年龄都比这些人大 经典型神经母细胞瘤(NB)，不分泌儿茶酚胺。所有肿瘤均为 NSE阳性，S-100蛋白50%阳性。EM的外观总体上是 更原始，但神经特征在大多数情况下是建立的。 单抗的免疫荧光显示出一种特殊的模式 PN染色，不同于NB和类似，尽管不同于 尤因肉瘤。

Peripheral neuroepithelioma (PN) has been underdiagnosed for many years, because of its histologic resemblance to Ewing's sarcoma in most cases, or occasionally to rhabdomyosarcomas (RMS). Lately, we were able to prove the neuroectodermal nature of the small round cell tumor of thoracopulmonary region ("Askin tumor"), a variant of PN, employing immunoperoxidase to detect the presence of the enzyme neuron-specific enolase (NSE) and electron microscopy (EM). In a wider study of small round cell tumors of childhood, we also found that NSE was only present in neuroectodermal tumors and not in Ewing's sarcoma or lymphoma. However, rhabdomyosarcomas (RMS) were also nonspecifically positive for NSE, and therefore, for distinction of RMS from PN, muscle markers and EM are additional diagnostic tools. Here we evaluated the histologic, immunocytochemical, cytochemical and EM features of 15 cases of PN and correlated them with clinical presentation. All tumors were extra-adrenal and occurred in diverse soft tissue and bony sites, in postganglionic locations. All patients were older than these in classic neuroblastoma (NB) and secreted no catecholamines. All tumors were NSE positive and 50% S-100 protein positive. The EM appearance was overall more primitive but the neural characters were established in most cases. Immunofluorescence with monoclonal antibodies showed a specific pattern of staining for PN, distinct from NB and similar, although not identical to Ewing's sarcoma.