ID #160 Diffuse Intrinsic Pontine Glioma in Children: Clinical and MRI Findings from a Low–Middle Income Country with an Illustrative Diffuse Midline Glioma Case
Raisul Maarif, Ahmad Data Dariansyah, Wihasto Suryaningtyas, Muhammad Arifin ParenrengiAbstract
Background
Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive pediatric brain tumor with a dismal prognosis. In low–middle income countries (LMICs), diagnosis is predominantly based on magnetic resonance imaging (MRI), while histological confirmation is infrequently performed. Diffuse midline glioma (DMG) may occasionally present with atypical imaging features, leading to discordance between radiological and pathological findings.
Methods
We conducted a retrospective single-center case series of pediatric patients with brainstem tumors. Patients fulfilling characteristic MRI criteria for DIPG—diffuse infiltrative pontine lesions with T2/FLAIR hyperintensity, with or without contrast enhancement—were included as the primary cohort. Clinical characteristics, treatment, and overall survival (OS) were analyzed. One additional patient with histologically confirmed diffuse midline glioma (DMG) and atypical MRI features was included as an illustrative case. OS was defined as the time from diagnosis to death or last follow-up.
Results
Five patients met MRI-based criteria for DIPG. Median age was 5 years. Common presenting symptoms included ataxia, hemiparesis, and cranial nerve palsies. Radiotherapy was administered in three patients. Median overall survival for MRI-based DIPG was approximately 8 months. Three patients died during follow-up, while two were alive at last follow-up. One additional patient with histologically confirmed DMG demonstrated atypical MRI features inconsistent with classic DIPG and died 2 months after diagnosis.
Conclusion
Pediatric DIPG in an LMIC setting continues to carry a poor prognosis, with MRI remaining the cornerstone of diagnosis. Histologically confirmed DMG may exhibit atypical imaging features, underscoring the importance of recognizing discordance between radiology and pathology. Clear distinction between DIPG and DMG is essential for accurate classification, reporting, and interpretation of outcomes.