Brain Tumors in Pediatrics

Resident Education Lecture Series

Brain Tumors - Background

      20-30% of cancers in children 2500-3000 new diagnoses/year 2 nd 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 Astrocytoma, low grade Astrocytoma, high grade Ependymoma Mixed glioma Ganglioglioma Oligodendroglioma PNET Choroid plexus tumor Meningioma Germ Cell Tumors Other 25-40% 8-20% 6-12% 2-5% 1-5% 1-5% 1-2% 1-2% 1-2% 1-2% 1-2% 1-3%

Location – Supra vs. Infra

 Infratentorial 45-60%       Medulloblastoma (PNET) Astrocytoma, low grade Ependymoma Brain stem glioma, high grade Brain stem glioma, low grade Other 20-25% 12-18% 4-8% 3-9% 3-6% 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  Ataxia  Tremors  Dysarthria  Stiff neck  Papilledema Brainstem primary  Extremity weakness  Cranial nerve signs – double vision – facial weakness – swallowing dysfunction

Hemispheric Tumors – Clinical Features

    Hemiparesis Hemianopsia Aphasia Seizures

Treatment

Tumor Type

Medulloblastoma Low grade astro cerebellar

Surgery

+++ +++ +++ optic glioma NO High grade astro/GBM +++ Brain stem glioma (exophytic) Ependymoma Germ cell tumor +++ ? bx

XRT

CrSp focal ????

????

+++ focal focal +++

Chemo

+++ --- --- ????

?

?

--- +++

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 PNET Pineoblastoma - posterior fossa - supratentorial - 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 of brain/spine, CSF  Prognostic factors: – Extent of resection!!!

– Age: some reports of better survival if > 5-7 years at diagnosis – Histology - MRI

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 – 2 nd 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