Dataset Information

High-grade glioma in infants and young children is histologically, molecularly, and clinically diverse: Results from the SJYC07 trial and institutional experience.

ABSTRACT:

Background

High-grade gliomas (HGG) in young children pose a challenge due to favorable but unpredictable outcomes. While retrospective studies broadened our understanding of tumor biology, prospective data is lacking.

Methods

A cohort of children with histologically diagnosed HGG from the SJYC07 trial was augmented with nonprotocol patients with HGG treated at St. Jude Children's Research Hospital from November 2007 to December 2020. DNA methylome profiling and whole genome, whole exome, and RNA sequencing were performed. These data were integrated with histopathology to yield an integrated diagnosis. Clinical characteristics and preoperative imaging were analyzed.

Results

Fifty-six children (0.0-4.4 years) were identified. Integrated analysis split the cohort into four categories: infant-type hemispheric glioma (IHG), HGG, low-grade glioma (LGG), and other-central nervous system (CNS) tumors. IHG was the most prevalent (n = 22), occurred in the youngest patients (median age = 0.4 years), and commonly harbored receptor tyrosine kinase gene fusions (7 ALK, 2 ROS1, 3 NTRK1/2/3, 4 MET). The 5-year event-free (EFS) and overall survival (OS) for IHG was 53.13% (95%CI: 35.52-79.47) and 90.91% (95%CI: 79.66-100.00) vs. 0.0% and 16.67% (95%CI: 2.78-99.74%) for HGG (p = 0.0043, p = 0.00013). EFS and OS were not different between IHG and LGG (p = 0.95, p = 0.43). Imaging review showed IHGs are associated with circumscribed margins (p = 0.0047), hemispheric location (p = 0.0010), and intratumoral hemorrhage (p = 0.0149).

Conclusions

HGG in young children is heterogeneous and best defined by integrating histopathological and molecular features. Patients with IHG have relatively good outcomes, yet they endure significant deficits, making them good candidates for therapy de-escalation and trials of molecular targeted therapy.

SUBMITTER: Chiang J

PROVIDER: S-EPMC10768990 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature

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Publications

High-grade glioma in infants and young children is histologically, molecularly, and clinically diverse: Results from the SJYC07 trial and institutional experience.

Chiang Jason J Bagchi Aditi A Li Xiaoyu X Dhanda Sandeep K SK Huang Jie J Pinto Soniya N SN Sioson Edgar E Dalton James J Tatevossian Ruth G RG Jia Sujuan S Partap Sonia S Fisher Paul G PG Bowers Daniel C DC Hassall Timothy E G TEG Lu Congyu C Zaldivar-Peraza Airen A Wright Karen D KD Broniscer Alberto A Qaddoumi Ibrahim I Upadhyaya Santhosh A SA Vinitsky Anna A Sabin Noah D ND Orr Brent A BA Klimo Paul P Boop Frederick A FA Ashford Jason M JM Conklin Heather M HM Onar-Thomas Arzu A Zhou Xin X Ellison David W DW Gajjar Amar A Robinson Giles W GW

Neuro-oncology 20240101 1

<h4>Background</h4>High-grade gliomas (HGG) in young children pose a challenge due to favorable but unpredictable outcomes. While retrospective studies broadened our understanding of tumor biology, prospective data is lacking.<h4>Methods</h4>A cohort of children with histologically diagnosed HGG from the SJYC07 trial was augmented with nonprotocol patients with HGG treated at St. Jude Children's Research Hospital from November 2007 to December 2020. DNA methylome profiling and whole genome, whol ...[more]

PMID: 37503880

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Dataset Information

High-grade glioma in infants and young children is histologically, molecularly, and clinically diverse: Results from the SJYC07 trial and institutional experience.

Background

Methods

Results

Conclusions

Publications

High-grade glioma in infants and young children is histologically, molecularly, and clinically diverse: Results from the SJYC07 trial and institutional experience.

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