Brain Tumors in Pediatrics

Resident Education
Lecture Series
Brain Tumors - Background

 20-30% of cancers in children

 2500-3000 new diagnoses/year
 2nd most common neoplasm
 Most occur before age 10 years
 Male/Female = 1.3/1.0
 60-70% 5 year survival
Relative Incidence of
Brain Tumors in Children

Table 25-1.
Approximate
incidence of
common CNS
tumors in children.
Pizzo & Poplack
Location – Supra vs. Infra

 Supratentorial 25-40%
 Astrocytoma, low grade 8-20%
 Astrocytoma, high grade 6-12%
 Ependymoma 2-5%
 Mixed glioma 1-5%
 Ganglioglioma 1-5%
 Oligodendroglioma 1-2%
 PNET 1-2%
 Choroid plexus tumor 1-2%
 Meningioma 1-2%
 Germ Cell Tumors 1-2%
 Other 1-3%
Location – Supra vs. Infra

 Infratentorial 45-60%

 Medulloblastoma (PNET) 20-25%

 Astrocytoma, low grade 12-18%
 Ependymoma 4-8%
 Brain stem glioma, high grade 3-9%
 Brain stem glioma, low grade 3-6%
 Other 2-5%
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
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?
 Posterior Fossa & Brainstem
Tumors - Clinical Features
Posterior Fossa primary Brainstem primary
 Ataxia  Extremity weakness
 Tremors  Cranial nerve signs
 Dysarthria – double vision
 Stiff neck – facial weakness
– swallowing
 Papilledema
dysfunction
Hemispheric Tumors –
Clinical Features
 Hemiparesis
 Hemianopsia
 Aphasia
 Seizures
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 +++ +++
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
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
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
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
Medulloblastoma
Prognostic Factors
 Age - Younger tend to do worse
 Extent of resection
 Non-posterior fossa tumors
 Non-localized disease
 Standard risk 70-80% 5 yr survival
High risk 50%

what are risk groups?

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?
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
Ependymoma - continued

Extent of resection most important

– Near to gross total resection 50-75%
– Less than NTR 0-30%
Radiation therapy helps survival
– Reduces local recurrence
Chemotherapy has not shown efficacy
Recurrence is rarely fixable
Brain Stem Gliomas

Diffuse intrinsic pontine 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
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
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
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
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
Credits

Sachin Jogal MD