Infant-type hemispheric glioma

Last revised by Daniel J Bell on 18 Apr 2022

Citation, DOI, disclosures and article data

Citation:

Buemi F, Bell D, Alsmair A, et al. Infant-type hemispheric glioma. Reference article, Radiopaedia.org (Accessed on 19 Apr 2024) https://doi.org/10.53347/rID-97352

DOI:

https://doi.org/10.53347/rID-97352

Permalink:

https://radiopaedia.org/articles/97352

rID:

97352

Article created:

10 Feb 2022, Francesco Buemi ◉

Disclosures:

At the time the article was created Francesco Buemi had no recorded disclosures.

View Francesco Buemi's current disclosures

Last revised:

18 Apr 2022, Daniel J Bell ◉

Disclosures:

At the time the article was last revised Daniel J Bell had no recorded disclosures.

View Daniel J Bell's current disclosures

Revisions:

4 times, by 4 contributors - see full revision history and disclosures

Systems:

Central Nervous System

Tags:

who cns 5, brain tumour, glioma

Synonyms:

Infant high-grade gliomas

Infant-type hemispheric gliomas, also known as infant high-grade gliomas, are high-grade brain tumors occurring in children.

Infant-type hemispheric gliomas belong to the family of "pediatric high-grade diffuse gliomas" of the 2021 WHO Classification of Tumors of the Central Nervous System ¹. They have been recently recognized as a novel tumor type distinct from other pediatric high-grade gliomas, harboring molecular mutations involving the receptor tyrosine kinases NTRK, ROS1, ALK, and MET.

Epidemiology

Infant-type hemispheric gliomas typically arise in the first year of life (range: 0–12 months) ².

Pathology

Subtypes

infant-type hemispheric glioma, NTRK-altered
infant-type hemispheric glioma, ROS1-altered
infant-type hemispheric glioma, ALK-altered
infant-type hemispheric glioma, MET-altered

Location

They are usually supratentorial ^2,3.

Microscopic appearance

Infant-type hemispheric gliomas show astrocytic cells arranged in fascicles or sheets with mild to moderate pleomorphism. Moreover, like the other high-grade gliomas, they often display palisading necrosis, mitotic activity, and endothelial proliferation ^2,3.

Immunophenotype and molecular alterations

GFAP: positive
ALK: sometimes positive in the infant-type hemispheric glioma ALK-altered
diagnosis of these tumors generally requires detection of the fusion genes of tyrosine kinase NTRK1, NTRK2, NTRK3, ROS1, ALK, MET or, alternatively, a characteristic methylation profile ^1-3

Radiographic features

An infant-type hemispheric glioma usually appears as a huge supratentorial mass with vasogenic edema, exerting a significant mass effect on the surrounding intracranial structures. Cases of leptomeningeal dissemination have been reported ⁴.

MRI

show a heterogeneous signal on T1W and T2W sequences, often with necrosis and hemorrhage
enhancement is present and is heterogeneous ^2,3

Treatment and prognosis

More studies are needed to evaluate the survival rate and impact of targeted therapies. Infant-type hemispheric glioma ALK-altered subtype seems to have a better 5-year survival rate than the ROS1- and NTRK-altered subtypes ².