Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update

被引：5

作者：

Ahluwalia, Ranbir ^{[1
,2
]}

Miles, Lili ^{[3
]}

Hayes, Laura ^{[4
]}

Scherer, Andrea ^{[5
]}

机构：

[1] Florida State Coll Med, 250 E Colonial Dr 200, Orlando, FL 32801 USA

[2] Florida State Univ, Coll Med, 1115 W Call St, Tallahassee, FL 32304 USA

[3] Nemours Childrens Hosp, Dept Pathol, Orlando, FL USA

[4] Nemours Childrens Hosp, Dept Neuroradiol, Orlando, FL USA

[5] Nemours Childrens Hosp, Dept Neurol Surg, Orlando, FL USA

来源：

CHILDS NERVOUS SYSTEM | 2020年 / 36卷 / 06期

关键词：

Septal dysembryoplastic neuroepithelial tumor (sDNT); Dysembryoplastic neuroepithelial tumor (DNT); Pediatric tumor; Ventricular tumor;

D O I：

10.1007/s00381-020-04581-y

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Purpose The purpose of this study was to review a case of a septal dysembryoplastic neuroepithelial tumor (sDNT) and compare it to cases reported in the current literature. Methods We review a case of sDNT and compare with 7 other previously noted cases in the literature. Results The mainstay treatment is gross total resection, and most patients achieve full clinical resolution. Septal dysembryoplastic neuroepithelial tumor (sDNT) is a rare pediatric disease most commonly presenting as intractable epilepsy or headache. sDNT has been recognized as a genotypically distinct entity from DNT. A high frequency (80%) of mutations of platelet-derived growth factor receptor A (PDGFRA) has been isolated in sDNT and could form the basis for future therapy. MRI is most commonly used to radiographically diagnose the disease and usually demonstrates a lobular interventricular mass involving the septum, potentially extending to the third ventricle. Conclusions Our case and literature review validates endoscopic biopsy as a diagnostic and therapeutic intervention.

引用

页码：1127 / 1130

页数：4

共 9 条

[1] Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: A lesion often misdiagnosed as glioma - Report of 10 cases [J].