Brain Tumors in Pediatrics - Medical College of Wisconsin

Report
Brain Tumors in Pediatrics
Resident Education
Lecture Series
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











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)
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
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







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
Surgery
Medulloblastoma
+++
Low grade astro
+++
cerebellar
+++
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
High risk
70-80% 5 yr survival
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
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
Credits
Sachin Jogal MD

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