Brain Tumors in Pediatrics

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Brain Tumors in Pediatrics
Resident Education Lecture Series
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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

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

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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

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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

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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?

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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

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Hemispheric Tumors Clinical Features

• Hemiparesis
• Hemianopsia
• Aphasia
• Seizures

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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

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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

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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

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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

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Medulloblastoma/PNET

• Similar histology, different tumor names based on
  location.
• Therapies vary
• Medulloblastoma - posterior fossa
• PNET - supratentorial
• Pineoblastoma - pineal region
• median age 5 years
• MF 21
• propensity to disseminate
• 1/3 with metastatic disease at diagnosis

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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?

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Medulloblastoma

• CSF dissemination
• check for leptomeningeal spread
• brain/spine MRI, LP
• Can spread to lung, liver, BM, bone, LNs rare
• Difference between supratentorial PNET (sPNET),
  medulloblastoma, and pineoblostoma?

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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 gt
  5-7 years at diagnosis
• Histology

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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

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Brain Stem Gliomas

• Diffuse intrinsic pontine gliomas
• median survival 6-9 months
• death within 2 years gt 90
• Radiation - transient clinical improvement
• Low grade gliomas
• tectal, exophytic, extra-medullary
• highly enhancing on MRI
• more indolent

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Low Grade Astrocytoma/Glioma

• 30-35 of CNS tumors
• 40-50 supratentorial, virtually anywhere
• MF 21
• Association with NF-1
• more indolent course
• GTR ? gt90 5 year survival
• RX
• Radiation
• Chemo if symptomatic, progressive, or recurrent

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Brain Tumors in lt 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

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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

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Pediatric Brain Tumors

• Leading cause of morbidity/mortality
  in pediatric cancers
• Need for aggressive supportive care
• Need for palliation

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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

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Credits

• Sachin Jogal MD