Abstract BACKGROUND AND OBJECTIVE The most commonly reported congenital intracranial space occupying lesions are teratoma, glioma and neuroblastoma. Primary intracranial congenital glioblastoma (PICG) is one of the most lethal and extremely rare neoplasms. Most pediatricians and gynecologists are unable to detect it congenitally, posing an ominous risk of spread and disease severity. We aim to determine the clinical manifestations, number of reported cases, radiological findings and neuro-oncological management of PICG. MATERIALS AND METHODS A comprehensive literature search using PubMed Central and Google Scholar was done, retrieving 17 case reports and five case series of 31 patients with PICG and included using the Preferred Reporting items for Systematic Reviews and Meta-Analyses guidelines, published between 2000 and 2023. RESULTS The mean age at presentation was 86.00 ± 88.36 days, with both genders affected equally, i.e. 45.16%. The most common symptoms and signs at presentation were seizures (38.70%) and hydrocephalus (51.61%), respectively. Left hemispheric (48.38%), with frontoparietal involvement (16.12%) was the frequently affected site. MRI findings were suggestive of low intensity signal significant of hemorrhage or calcification and a heterogenous mass in 35.58% and 32.25% cases, respectively. Gross total resection (GTR) was achievable in 48.38% patients. Glial fibrillary acid protein and vimentin were positive in 45.16% and 29.03% cases, respectively. Intraoperative mortality and recurrence were noted in 25.80% and 9.68% of patients, respectively. The average follow up duration was 20.57 months. CONCLUSION PICG has an extremely aggressive course of natural progression. Congenital presentation and its lack of awareness among clinicians is itself a challenge for disease management. Adjuvant chemo-radiotherapy has proven fruitful along with GTR. Post-operative CSF diversion is warranted.
Read full abstract