胚胎发育不良性神经上皮瘤病理形态学观察

Pathomorphologic features of dysembryoplastic neuroepithelial tumor

目的 探讨胚胎发育不良性神经上皮瘤 (DNT)临床病理特点、诊断及与低级别胶质细胞瘤鉴别诊断。方法 对 2例DNT和 5例少枝细胞瘤进行光镜、免疫组化和特殊染色观察。结果 2种肿瘤组织形态类似 ,主要由类似分化好少枝细胞瘤样细胞构成。与少枝细胞瘤不同 ,DNT的组织学特点是瘤组织黏液变明显 ,多见网状微囊变。瘤组织间散在分布分化好的神经细胞 ,多位于黏液丰富的微囊内 ,如同浮蛙。分化的神经细胞及部分小细胞Syn阳性。肿瘤邻近的大脑皮质神经细胞层次有不同程度紊乱。结论 DNT与低级别少枝胶质细胞瘤不易鉴别 ,DNT预后良好 ,只需手术切除即可 ,不需辅以有潜在危害的化疗或放疗。因此 ,两者的鉴别诊断具有重要的临床意义。

Purpose To investigate the clinicopathologic characteristics of dysembryoplastic neuroepithelial tumor (DNT)and the distinction with low grade oligodendrogliomas. Methods Compared with five oligodendrogliomas, two cases of DNT were analyzed with light microscopic observation, immunohistochamical stains and review of the literature. Results The histomorphology of DNT was similar with oligodendroglioma,and the main cells were well different oligodendroglioma like cells. Unlike oligodendrogliomas, however, DNT had obvious mucoid degeneration, and microcystic appearance. Well differentiated neurons were randomly scattered in microcystoid space (so called “floating” neurons). The differentiated neurons and some small cells presented positive for synaptophysin. Focal cerebr cortical dysplasia presented in neighborhood of the tumor. Conclusions The distinction between DNT and low grade oligodendroglioma may be difficult to make. It is important that both of the tumors are correctly distinguished at diagnosis, because patients with DNT need not be subjected to potentially harmful adjuvant therapies such as radiation or chemotherapy.