1. Brain Tumors in Pediatrics
Resident Education Lecture Series

2. Brain Tumors - Background
20-30% of cancers in children
new diagnoses/year
2nd most common neoplasm
Most occur before age 10 years
Male/Female = 1.3/1.0
60-70% 5 year survival

3. Relative Incidence of Brain Tumors in Children
Table Approximate incidence of common CNS tumors in children.
Pizzo & Poplack

6. Location – Supra vs. Infra
Supratentorial %
Astrocytoma, low grade %
Astrocytoma, high grade %
Ependymoma %
Mixed glioma %
Ganglioglioma %
Oligodendroglioma %
PNET %
Choroid plexus tumor %
Meningioma %
Germ Cell Tumors %
Other %

7. Location – Supra vs. Infra
Infratentorial %
Medulloblastoma (PNET) 20-25%
Astrocytoma, low grade %
Ependymoma %
Brain stem glioma, high grade 3-9%
Brain stem glioma, low grade 3-6%
Other %

8. Brain Tumors - Signs/Symptoms
Increased intracranial pressure - symptoms
Headache (am)
Nausea/vomiting (am)
Double vision
Head tilt
Decreased alertness
Lethargy/irritability
Poor feeding, FTT
Endocrine dysfunction
Unexplained behavior changes
- affect, motivation, energy level

9. Brain Tumors – Signs/Symptoms
Increased ICP – Signs
Papilledema, optic atrophy
Loss of vision
OFC (head circumference) increased
Bulging fontanelles, spreading sutures
“Setting sun” sign (Parinaud syndrome)
Increased blood pressure, low pulse  herniation?

10. Posterior Fossa & Brainstem Tumors - Clinical Features
Posterior Fossa primary
Ataxia
Tremors
Dysarthria
Stiff neck
Papilledema
Brainstem primary
Extremity weakness
Cranial nerve signs
double vision
facial weakness
swallowing dysfunction

11. Hemispheric Tumors – Clinical Features
Hemiparesis
Hemianopsia
Aphasia
Seizures

12. Treatment Tumor Type Surgery XRT Chemo Medulloblastoma +++ CrSp +++
Low grade astro focal
cerebellar ????
optic glioma NO ???? ????
High grade astro/GBM ?
Brain stem glioma (exophytic) focal ?
Ependymoma focal
Germ cell tumor ? bx

13. Treatment - Surgery In general, needed for diagnosis
- exceptions: GCT, BSG
Ideal is gross total resection
Balance prognosis vs. morbidity
Debulking, shunts, reservoirs
- for symptom/ICP reduction, therapy

14. Treatment – Radiation Therapy
Potential for use in all brain tumors
exceptions: choroid plexus tumors
Neuro-axis prophylaxis (cranio-spinal rx)
if tumor disseminates via CSF
Concerns for long term effects
neuro-cognitive
hearing
secondary cancers
endocrine
skeletal growth

15. Therapy - Chemotherapy
Adjunct therapy in most cases
particularly in GCT, medulloblastoma
Of interest in young children
(avoid or prolong XRT)
Blood brain barrier may be limiting
Newer studies suggest this may not be so
Local delivery via pumps/reservoir/IT

16. Medulloblastoma/PNET
Similar histology, different tumor names based on location.
Therapies vary
Medulloblastoma - posterior fossa
PNET - supratentorial
Pineoblastoma - pineal region
median age 5 years
M:F = 2:1
propensity to disseminate
1/3 with metastatic disease at diagnosis

17. Medulloblastoma Prognostic Factors
Age - Younger tend to do worse
Extent of resection
Non-posterior fossa tumors
Non-localized disease
Standard risk % 5 yr survival
High risk 50%
what are risk groups?

18. Medulloblastoma CSF dissemination
check for leptomeningeal spread
brain/spine MRI, LP
Can spread to lung, liver, BM, bone, LN’s – rare
Difference between supratentorial PNET (sPNET), medulloblastoma, and pineoblostoma?

19. Ependymoma 10% of childhood brain tumors Median age = 3-4 yrs
2/3 of primary in posterior fossa
May have leptomeningeal spread - MRI of brain/spine, CSF
Prognostic factors:
Extent of resection!!!
Age: some reports of better survival if > 5-7 years at diagnosis
Histology

20. Ependymoma - continued
Extent of resection most important
Near to gross total resection %
Less than NTR %
Radiation therapy helps survival
Reduces local recurrence
Chemotherapy has not shown efficacy
Recurrence is rarely fixable

21. Brain Stem Gliomas Diffuse intrinsic pontine gliomas Low grade gliomas
median survival = 6-9 months
death within 2 years > 90%
Radiation - transient clinical improvement
Low grade gliomas
tectal, exophytic, extra-medullary
highly enhancing on MRI
more indolent

23. Low Grade Astrocytoma/Glioma
30-35% of CNS tumors
40-50% supratentorial, virtually anywhere
M:F = 2:1
Association with NF-1
more indolent course
GTR  >90% 5 year survival RX
Radiation
Chemo if symptomatic, progressive, or recurrent

24. Brain Tumors in < 3 year olds
60-70% supratentorial
XRT has significant neuro-cognitive effects
Goal of therapies:
Delay XRT to at least 3 yrs old with chemotherapy  most relapse prior to XRT
Current study
Short course (16 wks) chemo
2nd look surgery
Focal (conformal) XRT
Maintenance chemotherapy

25. Complications From Tumor/Therapy
Neurological deficits
limb paresis
Rehab/PT/OT, support
swallowing/speech dysfunction
ENT, Speech therapy
Nutrition issues
neuro-cognitive deficits
School/education issues
Social interaction issues
endocrine dysfunction
end-organ damage
kidney, liver, hearing, neuropathy

26. Pediatric Brain Tumors
Leading cause of morbidity/mortality in pediatric cancers
Need for aggressive supportive care
Need for palliation

28. From ABP Certifying Exam Content Outline
Recognize the signs and symptoms of craniopharyngioma
Recognize the clinical manifestations of brain tumor
Recognize the physical characteristics of a headache due to increased intracranial pressure
Differentiate the clinical manifestations of spinal cord compression (eg, from a tumor) from those of other myelopathies, and evaluate appropriately

29. Credits
Sachin Jogal MD