PERIPHERAL NEUROEPITHELIOMA - A TUMOR DISTINCT FROM NEUROBLASTOMA

周围神经上皮瘤（PN）多年来一直被诊断不足， 因为在大多数情况下其组织学类似于尤文肉瘤，或 横纹肌肉瘤（RMS）。 最近，我们能够证明 胸部小圆细胞瘤的神经外胚层性质 区域（“Askin肿瘤”），PN的变体，采用免疫过氧化物酶， 检测神经元特异性烯醇化酶（NSE）的存在， 电镜（EM）。 在一项更广泛的研究中， 在儿童期，我们还发现NSE仅存在于神经外胚层， 而不是尤文肉瘤或淋巴瘤。 然而，横纹肌肉瘤 (RMS)NSE也呈非特异性阳性，因此， RMS与PN、肌肉标记物和EM的区别是额外的诊断 工具. 在此，我们评估了组织学，免疫细胞化学，细胞化学和EM 分析了15例PN患者的临床特征，并与临床表现进行了对照。 所有肿瘤均位于肾上腺外，发生于各种软组织和骨组织 在节后的位置。 所有患者年龄均大于这些， 典型的神经母细胞瘤（NB），不分泌儿茶酚胺。 所有肿瘤 NSE阳性，50%的S-100蛋白阳性。 EM外观总体上 更原始，但在大多数情况下，神经特征已经建立。 单克隆抗体的免疫荧光显示了一种特定的模式， PN染色，不同于NB和类似，但不完全相同 尤因氏肉瘤。

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.