Diffuse infiltrating pontine glioma (DIPG)

FACTS

  • WHO Grade IV glioma
  • part of a larger category of diffuse midline gliomas commonly occurring in:
    • pons
    • thalamus
    • spinal cord

HPI

universal ROS
  • loss of developmental milestones (age-dependent)
  • FTT
  • irritability
  • macrocephaly
  • gait difficulty
  • morning vomiting
  • triad of brainstem deficits
      1. CN deficits (facial weakness, eye movement disorders, swallowing dysfunction)
      1. hemibody motor/sensory deficits
      1. ataxia

PHYSICAL EXAM

universal exam
  • close attention to all cranial nerves
    • uvula position
    • shoulder shrug

IMAGING

MRI Brain w/wo contrast classic pattern
  • T1 hypo-intense, T1+c hypo-intense
  • T2 hyperintense

+ evaluate for basilar encasement
A/P
  • pan-neuro-axial imaging at the initial time of consultation to rule out drop mets
  • this is a life-changing diagnosis with grim prognosis, need a neuro-oncology consult for group conversation with family to appropriately deliver this heavy news
  • generally not operative except if
      1. needle biopsy needed for trial involvement / questionable diagnosis (3-5% risk of deficits)
      1. CSF diversion for hydrocephalus (ETV, EVD → shunt)
 

Young child with a classic DIPG

notion image