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
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