Diffuse Intrinsic Pontine Glioma (DIPG)
DIPG FACTS
- DIPG is 80% of all brainstem tumors (10-20% pediatric CNS tumors)
- Males = Females
- Age: 5-9 years
- One of 5 Brainstem glioma types
- DIPGs
- tectal (mesencephalic) tumors
- focal brainstemp tumors
- dorsal exophytic tumors
- cervicomedullary tumors
CONSULT
HPI
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FOCUSED EXAMClasses of Deficits 1. focal cranial neuropathies 2. cerebellar dysfunction 3. limb weakness (can involve corticospinal tracts) 4. +/- hydrocephalus (late-stage) Specific deficits - extraocular deficits - diplopia - bulbar signs: dysphagia, dysarthria - facial weakness - sensory loss - head tilting - dysmetria/dysdiadochokinesia |
IMG- evalauate for englufment of basilar artery +/- [ ] CTA head/neck - usually occupies majority of pons then extends into midbrain, medulla, peduncles - features c/w hypocellular tumor, usually 0-25% of tumor will enhance [ ] MRI Brain w/wo T1 / T2 / FLAIR / T1+c HYPO / HYPER / HYPER / minimal atypical features: prominent enhancement, decreased T2/FLAIR, diff restriction, exophytic components [ ] MRI spectroscopy: small decrease in N-acetylaspartate, increase in choline [ ] MRI perfusion HYPO-perfusion |
A/P- these are inoperable tumors - may be a role in tissue biopsy at most, for molecular therepeautics/investigational purposes [ ] No operative interevention +/- CSF diversion [ ] Radiation is primary treatment [ ] Counsel - this is a devastating diagnosis that must be carefully delivered to the family. Consider letting attending deliver news in AM. P&P Ch 42: "In most studies, the survival rate for DIPG is less than 10% at 2 years, with a median survival time of less than 1 year" - predictors of poor outcome: < 2yo, CN palsies (esp. CN 6), LTS, pontine location, basilar encasement, shorter sx duration - better outcome: older patients, neurofibromatosis, longer sx duration |
