Diffuse intrinsic pontine gliomas in adults: A retrospective study

Abstract

Background: Brainstem gliomas (BSG) constitutes very small proportion in adults brain tumors with pons as most common location. There is significant paucity in literature for adult diffuse intrinsic pontine gliomas (DIPG).

Objective: In this study, we attempt to review the outcomes of DIPG in single institute.

Methods: We performed a retrospective chart review of adult DIPG from last 8 years (2010-2018) in a tertiary institute. DIPG was defined as expansile lesions involving more than 50% of the greatest diameter in the pons.

Results: We found a total 46 patients with the diagnosis of adult BSG. Based on the definition, 23 patients with adult DIPG qualified to be included in the study. The median age was 32 years (IQR: 22-41), with a sex ratio of 16/7 (M/F). Cranial palsies were found in 17 (73%) patients. The median duration of symptoms was 6 months. On magnetic resonance imaging (MRI), contrast enhancement was found in seven (30%) patients. Biopsy was done in five patients. Median follow up was 11 months (IQR: 7-15). Median overall survival (OS) was 15 months (95%, CI 8.3-21.6). Fourteen patients had succumbed to death at the latest follow-up, and seven patients were alive. Median OS for the patients with age less than 40 years and more than 40 years was 7 and 22 months, respectively (p = 0.016). Rest of the variables did not effect OS significantly.

Conclusion: Adult DIPG's significantly differs from pediatric counterparts in clinical characteristics, as well as OS. Age was the only factor which was significantly associated with survival in our study. Long-term studies with molecular profiling may help in further characterizing these lesions.

Keywords: Brainstem gliomas; chemotherapy; diffuse intrinsic pontine gliomas; overall survival; radiotherapy.