Transcript Brain Tumors in Pediatrics - Medical College of Wisconsin
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
Medulloblastoma Low grade astro cerebellar
+++ +++ +++ optic glioma NO High grade astro/GBM +++ Brain stem glioma (exophytic) Ependymoma Germ cell tumor +++ ? bx
CrSp focal ????
+++ focal focal +++
+++ --- --- ????
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
be limiting – Newer studies suggest this may not be so – Local delivery via pumps/reservoir/IT
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?
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?
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
Sachin Jogal MD