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Brain Tumors in Pediatrics

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.

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Brain Tumors in Pediatrics

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  1. Brain Tumors in Pediatrics Resident Education Lecture Series

  2. 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

  3. Relative Incidence of Brain Tumors in Children Table 25-1. Approximate incidence of common CNS tumors in children. Pizzo & Poplack

  4. 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%

  5. 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%

  6. 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

  7. 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?

  8. Posterior Fossa primary Ataxia Tremors Dysarthria Stiff neck Papilledema Brainstem primary Extremity weakness Cranial nerve signs double vision facial weakness swallowing dysfunction Posterior Fossa & Brainstem Tumors - Clinical Features

  9. Hemispheric Tumors – Clinical Features • Hemiparesis • Hemianopsia • Aphasia • Seizures

  10. 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 +++ +++

  11. 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

  12. 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

  13. 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

  14. 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

  15. 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?

  16. 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?

  17. 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

  18. 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

  19. 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

  20. 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

  21. 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

  22. 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

  23. 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

  24. Credits • Sachin Jogal MD

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