Neuroepithelial tumors with embryogenesis dysplasia:a clinicopathological analysis of 33 cases
10.13315/j.cnki.cjcep.2024.05.006
- VernacularTitle:胚胎发育不良性神经上皮肿瘤33例临床病理分析
- Author:
Yi XIA
1
;
Shujing ZHENG
;
Ji XIONG
;
Zunguo DU
;
Xiaomu HU
;
Xing HU
;
Ying ZHANG
Author Information
1. 复旦大学附属华山医院病理科,上海 200040
- Keywords:
central nervous system tumors;
embryonic dysplas-tic neuroepithelial tumors;
differential diagnosis;
prognosis
- From:
Chinese Journal of Clinical and Experimental Pathology
2024;40(5):476-479
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To investigate the clinical,imaging,pathological features,differential diagnosis and prognosis of em-bryonic dysplastic neuroepithelial tumor(DNT).Methods The clinical data of 33 DNT patients were collected.Immunohis-tochemical results of Olig2,NeuN,Syn,GFAP,IDH1,CD34,BRAF V600E,H3K27M and Ki67 were examined by EnVision method.Histopathological morphology and immunohistochemical features were retrospectively analyzed and relevant literature was reviewed synchronously.Results A total of 28 cases were fol-lowed up,including 10 females and 18 males.The age of onset was 4～57 years,with average 24.97 years.Refractory epilepsy was a common symptom.The lesions were mainly located in the supratentorial cortex,and most of them were well-defined,lobu-lated,cystic or cystic and solid.Microscopically,DNT was mainly composed of oligodendrocytes,neurons floating in the mucous matrix,and proliferative astrocytes.Calcification was rare,and no necrosis or microvascular hyperplasia was ob-served.Immunohistochemistry showed that neurons expressed NeuN and Syn,oligodendrocytes expressed Olig2,and prolifera-tive astrocytes expressed GFAP;p53 was mostly wild-type,and the proliferation index of Ki67 was low(mostly less than 4％).Conclusion DNT is a benign tumor with mixed glial and neu-ronal structures.Precise pathological diagnosis needs to be care-fully considered with imaging characteristics,microscopic mor-phology,immunohistochemistry and molecular test results if nec-essary.Prognosis after complete surgical resection is good.
