结节型节细胞神经母细胞瘤3例并文献复习

A report of 3 cases of nodular ganglioneuroblastoma and the literature review

目的探讨结节型节细胞神经母细胞瘤(ganglioneuroblastoma-nodular,GNBn)的临床病理特征、免疫表型、诊断要点及鉴别诊断。方法对3例GNBn组织进行HE染色观察和免疫组织化学标记,收集临床资料,并复习相关文献。结果3例GNBn大体观均呈球形,包膜完整;HE染色镜下观察显示其由相互交织的神经纤维束、不同分化程度的神经母细胞和神经节细胞组成;免疫组织化学染色示神经节细胞及神经母细胞突触素(Syn)、嗜铬粒素A(CgA)、神经元特异性烯醇化酶(NSE)均呈阳性,核增殖指数高(Ki-67>40%)。结论GNBn是罕见的混合性肿瘤,极易被误诊,仔细的大体检查及镜下观察是诊断的关键,熟悉其临床病理学特点及预后判断对临床治疗具有指导意义。

Objective To investigate the clinicopathological features,immunophenotype,diagnosis points and differential diagnosis of nodular ganglioneuroblastoma(GNBn).Methods 3 cases of GNBn was analyzed by hematoxylin-eosin(HE)staining and immunohistochemistry,clinical data were collected,and relevant literature was reviewed.Results All 3 cases of GNBn were spherical in general with an intact envelope.Microscopically,tumor cells were composed of Schwannian stroma,neuroblasts,and differently differentiated gangliocytes.Immunohistochemically,both neuroblasts and gangliocytes were positive for synaptophysin(Syn),chromogranin A(CgA)and neuron-specific enolase(NSE)and the karyorrhexis index was high(Ki-67>40%).Conclusions GNBn is a rare mixed tumor which is easily misdiagnosed.Carefully gross examination and microscopic observation are the key points to diagnosis.Familiar with the clinicopathologic characteristics and prognostic evaluation of GNBn is of great significance to its clinical treatment.