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 primaryAtaxiaTremorsDysarthriaStiff neckPapilledema

Brainstem primaryExtremity weaknessCranial nerve signs

– double vision

– facial weakness

– swallowing 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 tumorsMedian age = 3-4 yrs2/3 of primary in posterior fossaMay have leptomeningeal spread -

MRI of brain/spine, CSFPrognostic 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 efficacyRecurrence 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 = 2:1Association with NF-1

– more indolent courseGTR >90% 5 year survivalRX

– Radiation– Chemo if symptomatic, progressive, or recurrent

Brain Tumors in < 3 year olds

60-70% supratentorialXRT has significant neuro-cognitive effectsGoal 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