Infantile atypical subependymal giant cell astrocytoma

Subependymal giant cell astrocytoma is a benign WHO grade I intraventricular tumor arise in patients with tuberous sclerosis complex. Previous reported described histopathological predictors of more aggressive forms, terms atypical SEGA in infantile age group. Other reports showed possible transform...

Full description

Saved in:
Bibliographic Details
Published in:Neurosciences (Riyadh, Saudi Arabia) Saudi Arabia), 2020-01, Vol.25 (1), p.61-64
Main Authors: Almubarak, Abdulaziz O, Abdullah, Jamal, Al Hindi, Hindi, AlShail, Essam
Format: Article
Language:English
Subjects:
Case Report
Gliomas
Tuberous sclerosis
Vincristine
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Subependymal giant cell astrocytoma is a benign WHO grade I intraventricular tumor arise in patients with tuberous sclerosis complex. Previous reported described histopathological predictors of more aggressive forms, terms atypical SEGA in infantile age group. Other reports showed possible transformation of SEGA into glioblastoma, or misdiagnosis as glioblastoma due to the presence of atypical histopathological features. Here, we report a case of an infant who presented with right frontal extraventricular SEGA and underwent craniotomy with complete resection. Eight months later, he presented with fast recurrence in same location with midline shift and subfalcine herniation. Histopathological description showed high grade features including Ki labeling index of 60%, atypical mitotic figures, cellular plemorphism and necrosis. We also discussed the possible presence of different entity (termed atypical SEGA) which may have more aggressive clinical course, with literature review of predictors of SEGA aggressiveness and possible transformation/misdiagnosis as glioblastoma.
ISSN:1319-6138
1319-6138
DOI:10.17712/nsj.2020.1.20190044