关键词: 先天性神经母细胞瘤, 囊性, 肾上腺, PHOX2B

Abstract: 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 PHOX2B,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.

Key words: congenital neuroblastoma, cystic, adrenal, PHOX2B