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目的探讨结节型节细胞性神经母细胞瘤(GNBn)的临床、病理形态学特点、诊断与鉴别诊断及预后。方法应用与年龄相关的危险因素分组方法,分析10例GNBn的临床表现、影像学、组织学特点。结果 10例GNBn患儿,<1.5岁1例,1.5～5岁6例,>5岁3例,平均年龄4.3岁。肿物位于肾上腺7例,后纵隔3例。按照Evan’s分期,Ⅱ期2例,Ⅲ期2例,Ⅳ期6例。10例中3例肉眼可见瘤结节,4例因肿瘤钙化、坏死掩盖了结节存在,另3例大体检查与神经母细胞瘤类似;镜下9例瘤结节为分化差的神经母细胞瘤,1例为分化型,其中3例见大量核碎裂细胞;8例瘤结节周围为节细胞性神经瘤,2例为混杂型节细胞性神经母细胞瘤。危险因素分组:1例属预后良好的组织学类型,9例属预后不良的组织学类型。结论结节型节细胞性神经母细胞瘤具有独特的临床、病理特点,大体及镜下仔细检查是诊断的关键;与神经母细胞瘤类似,也可分为预后良好和预后不良的组织学类型。
Objective To investigate the clinicopathological features, diagnosis, differential diagnosis and prognosis of nodular ganglioneuroblastoma (GNBn). Methods The age-related risk factors grouping method was used to analyze the clinical manifestations, imaging and histological features of 10 cases of GNBn. Results 10 cases of GNBn children, <1.5 years in 1 case, 1.5 to 5 years in 6 cases,> 5 years old in 3 cases, mean age 4.3 years. Tumor in the adrenal in 7 cases, 3 cases after the mediastinum. According to Evan’s staging, there were 2 cases in stage II, 2 cases in stage III and 6 cases in stage IV. Tumor nodules were seen in 3 of the 10 cases. Nodules were found in 4 cases due to tumor calcification and necrosis. The other 3 cases were similar to neuroblastoma in general examination. Among the 9 cases, poorly differentiated neuroblasts One of them was differentiated, of which 3 were found in a large number of nuclear fragmented cells. Eight of the nodules were ganglioneuropathies and two were ganglioneurous neuroblastomas. Risk factors grouping: 1 case of a good prognosis histological type, 9 cases of poor prognosis histological type. Conclusions Nodular ganglioneuroblastoma has unique clinical and pathological features. The gross and microscopic examination is the key to diagnosis. Similar to neuroblastoma, it can be divided into histological type with good prognosis and poor prognosis .