先天性神经母细胞瘤六例临床病理分析

Clinicopathological analysis of 6 cases of congenital neuroblastoma

目的探讨先天性神经母细胞瘤的临床病理特征、免疫表型、分子机制、诊断及鉴别诊断。方法回顾性分析6例先天性神经母细胞瘤临床表现、病理特征、分子机制等,并随访及文献复习。结果6例病人中,囊性4例,实性2例。发病部位为肾上腺5例,后纵隔1例。镜下病理特征为肿瘤细胞被纤细纤维分割成巢团状,肿瘤细胞小而圆形,染色质细腻,可有小核仁。免疫组化PHOX2B、Syn、CgA及CD56染色均(+)。结论先天性神经母细胞瘤发病率低,生物学行为具有异质性,可自发消退或分化为良性的神经胶质瘤等,也可迅速恶化、转移迅速进展,分析其临床病理特征有助于深入了解该肿瘤的治疗与预后。

Objective To explore the clinicopathological features,immunophenotype,molecular mechanism,diagnosis and differential diagnosis of congenital neuroblastoma.Methods The clinical manifestations,pathological features,and molecular mechanisms of 6 cases of congenital neuroblastoma were retrospectively analyzed,followed by telephone follow-up and literature review.Results Among the 6 patients,4 were cystic and 2 were solid.Five cases were adrenal glands and one case was posterior mediastinum.The pathological feature under the microscope is that the tumor cells are divided into nests by fine fibers,the tumor cells are small and round,the chromatin is fine,and there may be small nucleoli.Immunohistochemistry showed PHOX2 B,Syn,CgA and CD56 were all positive.Conclusion The incidence of congenital neuroblastoma is low,and the biological behavior is heterogeneous.It can regress spontaneously or differentiate into a benign glioma.It can also rapidly deteriorate and metastasize rapidly.Analysis of its clinicopathological features helps In-depth understanding of the treatment and prognosis of this tumor.