Response assessment in diffuse intrinsic pontine glioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group

Lancet Oncol. 2020 Jun;21(6):e330-e336. doi: 10.1016/S1470-2045(20)30166-2.


Optimising the conduct of clinical trials for diffuse intrinsic pontine glioma involves use of consistent, objective disease assessments and standardised response criteria. The Response Assessment in Pediatric Neuro-Oncology working group, consisting of an international panel of paediatric and adult neuro-oncologists, clinicians, radiologists, radiation oncologists, and neurosurgeons, was established to address issues and unique challenges in assessing response in children with CNS tumours. A working group was formed specifically to address response assessment in children and young adults with diffuse intrinsic pontine glioma and to develop a consensus on recommendations for response assessment. Response should be assessed using MRI of brain and spine, neurological examination, and anti-inflammatory or antiangiogenic drugs. Clinical imaging standards are defined. As with previous consensus recommendations, these recommendations will need to be validated in prospective clinical trials.

Publication types

  • Review

MeSH terms

  • Age of Onset
  • Brain Stem Neoplasms / diagnostic imaging*
  • Brain Stem Neoplasms / epidemiology
  • Brain Stem Neoplasms / pathology
  • Brain Stem Neoplasms / therapy*
  • Diffuse Intrinsic Pontine Glioma / diagnostic imaging*
  • Diffuse Intrinsic Pontine Glioma / epidemiology
  • Diffuse Intrinsic Pontine Glioma / pathology
  • Diffuse Intrinsic Pontine Glioma / therapy*
  • Endpoint Determination / standards*
  • Humans
  • Magnetic Resonance Imaging / standards*
  • Neoplasm Grading
  • Neuroimaging / standards*
  • Predictive Value of Tests
  • Time Factors
  • Treatment Outcome
  • Tumor Burden