Primary Intracranial High Grade Congenital Glial Lesions (PIHGCL): A systematic review of case reports from 1985 to 2025.

Abstract / Summary

Among congenital intracranial space-occupying lesions, teratomas, gliomas, and neuroblastomas are more frequently reported. However, primary congenital glioblastoma (cGBM) and congenital anaplastic astrocytoma (cAA) are underrecognized entities. Their detection remains challenging due to nonspecific prenatal findings and limited awareness among pediatricians and obstetric care providers, contributing to delayed diagnosis and worsened outcomes. To summarize the clinical presentations, diagnostic imaging characteristics, reported cases, histopathological features, and neuro-oncological management strategies of primary intracranial high grade congenital glial lesions (PIHGCL). A comprehensive literature search following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was conducted in 2025 over PubMed and Google Scholar using keywords and Boolean operators, retrieving 35 studies (44 patients) with PIHGCL published 1985 to 2025. Quality assessment was done using Joanna Briggs Institute (JBI) tool. The data was analyzed descriptively as well as analytically to determine survival outcomes in treatment groups. A total of 28 studies (37 patients) discussed cGBM, and 7 studies (7 patients) described the occurrence of cAA. Most of the patients were infants i.e., 65.9% (29), presenting with macrocephaly in 27.7% (10) patients. Most of the lesions were intracerebral, 72.7% (32), lateralized equally to both sides, i.e., 46.5% (20). Surgical excision alone was done in 29.5% (13) patients, surgical excision with adjuvant chemotherapy was given to 43.2% (19) patients, chemotherapy alone in 6.8% (3) patients whereas 20.5% (9) underwent only conservative treatment. Kaplan Meir-analysis revealed a significant difference in survival in patients who received adjuvant treatment along with surgical excision in contrast to other treatment groups (χ2 = 7.83, df = 3, p = 0.05). PIHGCL presents during fetal or neonatal period with clinicopathological features distinct from adult and pediatric glioblastomas with distinct genetic etiology. Extent of resection is not a reliable marker of treatment outcomes in PIHGCL due to anatomical and physiological variations at the age of presentations. Adjuvant chemotherapy along with surgical resection results in improved patient survival.

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