Diffuse intrinsic pontine gliomas in adults: A retrospective study

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.

[1]  M. van Veelen,et al.  Biopsy in diffuse pontine gliomas: expert neurosurgeon opinion—a survey from the SIOPE brain tumor group , 2020, Child's Nervous System.

[2]  T. Merchant,et al.  Radiation dose response of neurologic symptoms during conformal radiotherapy for diffuse intrinsic pontine glioma , 2020, Journal of Neuro-Oncology.

[3]  S. Browd,et al.  Progress in diffuse intrinsic pontine glioma: advocating for stereotactic biopsy in the standard of care. , 2020, Neurosurgical focus.

[4]  David T. W. Jones,et al.  Brainstem biopsy in pediatric diffuse intrinsic pontine glioma in the era of precision medicine: the INFORM study experience. , 2019, European journal of cancer.

[5]  K. Srinivasan,et al.  Diffusion tensor imaging and tractography in diffuse intrinsic pontine glioma – A major determinant of resectability and description of a new subtype , 2019, Neurology India.

[6]  H. Park,et al.  Developing chemotherapy for diffuse pontine intrinsic gliomas (DIPG). , 2017, Critical reviews in oncology/hematology.

[7]  B. Devi,et al.  Isolated brainstem tuberculomas , 2017, Acta Neurochirurgica.

[8]  Young Zoon Kim,et al.  A Case of Primary Central Nervous System Lymphoma Located at Brain Stem in a Child , 2016, Brain tumor research and treatment.

[9]  D. Reardon,et al.  Adult brainstem gliomas , 2016, Cancer.

[10]  S. Kesari,et al.  Brainstem Glioma in Adults , 2016, Front. Oncol..

[11]  Hai Yan,et al.  The H3.3 K27M mutation results in a poorer prognosis in brainstem gliomas than thalamic gliomas in adults. , 2015, Human pathology.

[12]  K. Aldape,et al.  Adult brainstem gliomas: Correlation of clinical and molecular features , 2015, Journal of the Neurological Sciences.

[13]  N. Martin-Duverneuil,et al.  Temozolomide after radiotherapy in recurrent “low grade” diffuse brainstem glioma in adults , 2014, Journal of Neuro-Oncology.

[14]  R. McLendon,et al.  Malignant brainstem gliomas in adults: clinicopathological characteristics and prognostic factors , 2014, Journal of Neuro-Oncology.

[15]  R. Stupp,et al.  Clinical management and outcome of histologically verified adult brainstem gliomas in Switzerland: a retrospective analysis of 21 patients , 2014, Journal of Neuro-Oncology.

[16]  M. Trippel,et al.  Retrospective analysis of 104 histologically proven adult brainstem gliomas: clinical symptoms, therapeutic approaches and prognostic factors , 2014, BMC Cancer.

[17]  N. Wetjen,et al.  Histologically Proven, Low-grade Brainstem Gliomas in Children: 30-Year Experience With Long-term Follow-up at Mayo Clinic , 2014, American journal of clinical oncology.

[18]  A. Friedman,et al.  Clinical characteristics and treatment of malignant brainstem gliomas in elderly patients , 2013, Journal of Clinical Neuroscience.

[19]  S. Blond,et al.  Correlation between magnetic resonance imaging findings and histological diagnosis of intrinsic brainstem lesions in adults. , 2012, Neuro-oncology.

[20]  Ryung S. Kim,et al.  Prognostic factors in adult brainstem gliomas: a multicenter, retrospective analysis of 101 cases , 2008, Journal of Neuro-Oncology.

[21]  A. Bizzi,et al.  Natural history and management of brainstem gliomas in adults , 2008, Journal of Neurology.

[22]  D. Hargrave,et al.  Diffuse brainstem glioma in children: critical review of clinical trials. , 2006, The Lancet. Oncology.

[23]  J J Mazeron,et al.  Brainstem gliomas in adults: prognostic factors and classification. , 2001, Brain : a journal of neurology.

[24]  L. Deangelis,et al.  Adult brainstem gliomas , 1998, Neurology.

[25]  Roger J. Packer,et al.  Magnetic resonance scans should replace biopsies for the diagnosis of diffuse brain stem gliomas: a report from the Children's Cancer Group. , 1993, Neurosurgery.

[26]  V. Rajshekhar,et al.  Brainstem Glioma: Comparative Study of Clinico-Radiological Presentation, Pathology and Outcome in Children and Adults , 1999, Acta Neurochirurgica.

[27]  R. Abbott,et al.  Intrinsic tumors of the medulla: predicting outcome after surgery. , 1996, Pediatric neurosurgery.

[28]  M. Prados,et al.  Hyperfractionated irradiation for adults with brainstem gliomas. , 1991, International journal of radiation oncology, biology, physics.