乳头状胶质神经元肿瘤临床病理分析

目的　报道 2例罕见的乳头状胶质神经元肿瘤 (PGNT) ,结合文献探讨其临床病理学特征。方法　对 2例PGNT进行临床病理分析及免疫组化观察。结果　 2例均为青年患者 ,临床表现为发作性头痛或慢性难治性癫 ,神经系统检查均无局灶性定位体征 ,MRI检查见颞叶脑实质内有边缘清晰的囊性病灶 ,囊壁内附有实性结节。镜下见肿瘤由神经元和胶质成分混合构成 ,并呈特征性的乳头结构 ,乳头中心为透明样变的血管 ,乳头表面为单层GFAP阳性的胶质细胞 ,另见乳头之间或成片排列的小圆形Syn阳性的神经元分化的细胞 ,散在有神经节样细胞和 或神经节细胞 ,未见坏死和核分裂象。结论　PGNT是一种新的独特的神经元 -胶质混合性肿瘤 ,预后良好

Papillary glioneuronal tumor: a clinicopathological study

Objective To report 2 cases of papillary glioneuronal tumor and to describe its clinicopathologic features with review of the literature. Methods The clinicopathologic data and immunohistochemical findings of PGNT were analyzed. Results Both of the two patients were young adults. One presented with discontinous headaches, and the other exhibited intractable seizures. No focal neurologic deficits were observed. Magnetic resonance imaging showed demarcated cystic lesion with mural nodule in the temporal lobe. Histopathological examination revealed a pseudopapillary component composed of hyalinized vessels covered by a single layer of GFAP-positive astrocytes. The Syn-positive small round neuronal component filled the interstices between pseudopapillary or formed solid sheets with ganglion-like cells or/and ganglion cells in it. Neither mitoses nor necrosis were seen. Conclusion PGNT is a new varient of mixed neuronal-glial neoplasm and has a favorable outcome.