The Trends in Diagnosis, Management and Care of Patients with Diffuse Intrinsic Pontine Gliomas: Perspectives from a Tertiary Care Hospital of Pakistan

Background: Diffuse Intrinsic Pontine Glioma (DIPG) primarily affects pediatric patients. Data on the global incidence of DIPG remain sparse, especially in South Asia and low-middle-income countries like Pakistan. Methods: After exemption from the Ethics Review Committee, a retrospective study was conducted. Records of patients with DIPG at the Aga Khan Hospital in Karachi, from January 2010 to December 2022, were reviewed. Results: A total of 35 pediatric patients were diagnosed/treated for DIPG. The median age of the patients was 9, with 19 (54.3%) males and 16 (45.7%) females. Cranial nerve palsies were the most common complaint in 19 (54.3%) patients, followed by headaches in 18 (51.4%), long tract signs in 14 (40%), ataxia/cerebellar symptoms in 14 (40%), and seizures in 5 (14.3%). MRI was the primary diagnostic tool, used alone or with CT in 32 (94.1%) patients; CT alone was used in 2 (5.7%). Biopsy was performed in 10 (28.6%) patients. Primary radiation therapy was administered to 14 (40%) patients with 5400 cGy in 30 fractions. All these patients received steroids. No patients received reirradiation. VP shunt surgery for hydrocephalus was performed in 9 (25.7%) patients. Over half (54.3%) refused treatment post-diagnosis, and 71.4% were lost to follow-up. Conclusion: Providing timely, quality multi-disciplinary care to DIPG patients within resource constraints remains challenging in Pakistan. However, recent developments show promise for improving DIPG care in the country.