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Founded in 1998, Amrita Hospitals provide a comprehensive spectrum
of general and specialist medical services as well as cross-
specialty consultation.
 Over 10 lakh + outpatients annually
 Over 70,000 + inpatients annually
 3.3 million square feet of built-up area
 740 faculty members
 275 equipped intensive-care bed
 Digital radiology department
 24/7 telemedicine service
 Fully computerized and networked
hospital information system
1,300-bed tertiary referral
and teaching hospital
100000+ Treated more than 10
million (1 crore) patients across India
31 modern operation
theatres
24 super specialty
departments
125 acres of greenery 4500 support staff
Amrita Hospital, Faridabad - Mission
Amrita Hospitals are world-class healthcare facilities and
Centres of Excellence dedicated to improving the well-being of
communities through quality programs of preventive and
curative medicine, medical education, and research. We are
committed to provide outstanding and affordable medical care in
a patient-friendly environment and in a spirit of compassion to
all, regardless of race, caste, religion, or economic condition.
Dr Anurag Sharma
Consultant Pediatric Neurosurgeon
Amrita Hospital, Faridabad
Brain Tumors in Pediatrics
4
Dr Sachin Gupta
Consultant Pediatric Neurosurgeon
Amrita Hospital, Faridabad
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
Pediatric Brain Tumors – Relative
Incidence
Low grade
High grade
Pineal tumors
Medulloblastoma
Cerebellar
astrocytoma
Ependymoma
Brain stem glioma
Other
Craniopharyngioma
15-25%
10-15%
(0.5-2%)
10-20%
10-20%
10-20%
5-10%
12-14%
From:.Approximate incidence of
common CNS tumors in children.
Pizzo & Poplack
0
10
20
30
40
50
60
70
80
90
A-supratentorial
C-infratentorial
Adult Child
Brain Tumors : Distribution
Brain Tumors : Supra- vs Infra-
tentorial
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%
 Others 1-3%
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%
 Others 2-5%
Brain Tumors : Supra- vs Infra-
tentorial
Brain Tumors : Bimodal
Distribution
http:/ / seer.cancer.gov/ statfacts/ html/ brain.html
and women per year. These rates are age-adjusted and based on 2006-2
Lifetime Risk
Lifetime Risk: Lifetime risk is the probability of developing or dying from
lifespan. Based on the most recent data, approximately 0.6 percent of me
brain and other nervous system cancer at some point during their lifetime
Prevalence of this cancer
Prevalence of this cancer: In 2010, there were an estimated 141,553
nervous system cancer in the United States.
Brain Tumors : 5 year Survival
Brain Tumors – Signs / Symptoms
 Increased intracranial pressure - symptoms
 Headache (am)
 Nausea/vomiting (am)
 Double vision
 Head tilt
 Decreased alertness
 Lethargy/irritability
 Poor feeding, Failure to thrive
 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 Tumors : C/F
 Ataxia
 Tremors
 Dysarthria
 Stiff neck
 Papilledema
Brainstem Primary Tumors : C/F
Extremity weakness
Cranial nerve signs
double vision
facial weakness
swallowing dysfunction
Hemispheric Tumors : C/F
 Hemiparesis
 Hemianopsia
 Aphasia
 Seizures
Brain Tumors – 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
Treatment – 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% 5 yr survival
Standard Risk - Age>3, Residual tumor <1.5cc, M0, Desmoplastic, No brain
stem invasion, low mitotic index, diploid DNA, high apoptotic index
High Risk - Age<3, Residual tumor>1.5cc, M1, Anaplastic, Brain stem invasion, high
mitotic index, Aneuploid DNA, low apoptotic index
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 - MRI of brain/spine,
CSF
 Prognostic factors:
 Extent of resection!!!
 Age: some reports of better survival if > 5-7 years at
diagnosis
 Histology
Ependymoma
 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
Brainstem Gliomas
 Diffuse intrinsic pontine gliomas (DIPG)
 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
Brainstem Gliomas
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 yr 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 – Tumor / Therapy
 Neurological deficits
 limb paresis
 Rehab/PT/OT, support
 swallowing/speech dysfunction
 ENT, Speech therapy
 Nutrition issues
 Neuro-cognitive deficits
 School/Education / Social interaction issues
 Endocrine dysfunction
 End-organ damage
 kidney, liver, hearing, neuropathy
Pediatric Brain Tumors - Summation
 Leading cause of morbidity/mortality in pediatric cancers
 Need for aggressive supportive care
 Need for palliation
 Rehabilitation is important part of treatment
Adult & Pediatric Neurosurgery Team
Thank You

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Management of Brain Tumors in Pediatric Age Group.pptx

  • 1.
  • 2. Founded in 1998, Amrita Hospitals provide a comprehensive spectrum of general and specialist medical services as well as cross- specialty consultation.  Over 10 lakh + outpatients annually  Over 70,000 + inpatients annually  3.3 million square feet of built-up area  740 faculty members  275 equipped intensive-care bed  Digital radiology department  24/7 telemedicine service  Fully computerized and networked hospital information system 1,300-bed tertiary referral and teaching hospital 100000+ Treated more than 10 million (1 crore) patients across India 31 modern operation theatres 24 super specialty departments 125 acres of greenery 4500 support staff
  • 3. Amrita Hospital, Faridabad - Mission Amrita Hospitals are world-class healthcare facilities and Centres of Excellence dedicated to improving the well-being of communities through quality programs of preventive and curative medicine, medical education, and research. We are committed to provide outstanding and affordable medical care in a patient-friendly environment and in a spirit of compassion to all, regardless of race, caste, religion, or economic condition.
  • 4. Dr Anurag Sharma Consultant Pediatric Neurosurgeon Amrita Hospital, Faridabad Brain Tumors in Pediatrics 4 Dr Sachin Gupta Consultant Pediatric Neurosurgeon Amrita Hospital, Faridabad
  • 5. 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
  • 6. Pediatric Brain Tumors – Relative Incidence Low grade High grade Pineal tumors Medulloblastoma Cerebellar astrocytoma Ependymoma Brain stem glioma Other Craniopharyngioma 15-25% 10-15% (0.5-2%) 10-20% 10-20% 10-20% 5-10% 12-14% From:.Approximate incidence of common CNS tumors in children. Pizzo & Poplack
  • 8. Brain Tumors : Supra- vs Infra- tentorial 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%  Others 1-3%
  • 9. 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%  Others 2-5% Brain Tumors : Supra- vs Infra- tentorial
  • 10. Brain Tumors : Bimodal Distribution http:/ / seer.cancer.gov/ statfacts/ html/ brain.html and women per year. These rates are age-adjusted and based on 2006-2 Lifetime Risk Lifetime Risk: Lifetime risk is the probability of developing or dying from lifespan. Based on the most recent data, approximately 0.6 percent of me brain and other nervous system cancer at some point during their lifetime Prevalence of this cancer Prevalence of this cancer: In 2010, there were an estimated 141,553 nervous system cancer in the United States.
  • 11. Brain Tumors : 5 year Survival
  • 12. Brain Tumors – Signs / Symptoms  Increased intracranial pressure - symptoms  Headache (am)  Nausea/vomiting (am)  Double vision  Head tilt  Decreased alertness  Lethargy/irritability  Poor feeding, Failure to thrive  Endocrine dysfunction  Unexplained behavior changes - affect, motivation, energy level
  • 13. 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?
  • 14. Posterior Fossa Tumors : C/F  Ataxia  Tremors  Dysarthria  Stiff neck  Papilledema
  • 15. Brainstem Primary Tumors : C/F Extremity weakness Cranial nerve signs double vision facial weakness swallowing dysfunction
  • 16. Hemispheric Tumors : C/F  Hemiparesis  Hemianopsia  Aphasia  Seizures
  • 17. Brain Tumors – 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 +++ +++
  • 18. 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
  • 19. 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
  • 20. Treatment – 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
  • 21. 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
  • 22. 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% 5 yr survival Standard Risk - Age>3, Residual tumor <1.5cc, M0, Desmoplastic, No brain stem invasion, low mitotic index, diploid DNA, high apoptotic index High Risk - Age<3, Residual tumor>1.5cc, M1, Anaplastic, Brain stem invasion, high mitotic index, Aneuploid DNA, low apoptotic index
  • 23. 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?
  • 24. 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
  • 25. Ependymoma  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
  • 26. Brainstem Gliomas  Diffuse intrinsic pontine gliomas (DIPG)  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
  • 28. 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
  • 29. Brain Tumors in <3 yr 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
  • 30. Complications – Tumor / Therapy  Neurological deficits  limb paresis  Rehab/PT/OT, support  swallowing/speech dysfunction  ENT, Speech therapy  Nutrition issues  Neuro-cognitive deficits  School/Education / Social interaction issues  Endocrine dysfunction  End-organ damage  kidney, liver, hearing, neuropathy
  • 31. Pediatric Brain Tumors - Summation  Leading cause of morbidity/mortality in pediatric cancers  Need for aggressive supportive care  Need for palliation  Rehabilitation is important part of treatment
  • 32. Adult & Pediatric Neurosurgery Team