Download presentation
Presentation is loading. Please wait.
1
Pediatric Brain Tumors
Corey Raffel, M.D., Ph.D. Chief, Section of Pediatric Neurosurgery Nationwide Children’s Hospital Columbus, Ohio
2
Brain Tumors in Children
“Oh, my God, my child/patient has a brain tumor!” Depression!
3
Brain Tumors in Children
What are the symptoms and signs of brain tumors in children? What kinds of tumors are there and how are they treated? Is the outcome always so bad?
4
Symptoms and Signs Headache. When to think about getting a scan.
Easy, if headache is associated with neurologic symptoms or signs. Lethargy, personality change, clumsiness of hands or gait, diplopia, bradycardia, hypertension Papilledema! Must see the fundus.
5
Symptoms and Signs Hard when associated neurologic signs are mild or absent. Nocturnal awakening with headache or headache at awakening in morning Vomiting associated with headache, often in the morning, often with temporary relief afterwards
6
Symptoms and Signs Headache worsened by lying down, coughing, laughing, Valsalva Posterior location of the headache Distinct onset less than 6 months or distinct change in pattern of pre-existing headache Progressive worsening over time
7
Symptoms and Signs Vomiting when associated with headache
When occurs in the morning, but not later in the day When new, interfering with food intake
8
Symptoms and Signs Seizures
New onset seizure unassociated with fever Change in pattern of pre-existing seizures
9
Symptoms and Signs In the infant, things are even harder.
Loss of previously acquired motor milestones Accelerated head growth after a period of normal growth Feel for full fontanelle, separated sutures
10
Imaging Studies These days, MR imaging is the modality of choice. Better at delineating tumors. No irradiation. Scan without and with contrast is best Quick MR scan? Axial T2-weighted images can be used as a screen. Quick and cheaper.
11
Types of Tumors Brain tumors is children are not common, about 3 cases per 100,000 children less than 15 years of age per year or about 1500 new cases per year. This means that about 1 in every 2700 such children will get a brain tumor. But brain tumors are common amongst types of tumors that children get, second only to leukemia in both incidence and mortality.
12
Types of Tumors Will review the common tumor types, emphasizing presentation, treatment, and outcome.
13
Medulloblastoma/PNET
PNET describes morphologically related CNS tumors Unrelated to PNS PNET characterized by translocation Medulloblastoma=cerebellar PNET PNET terminology being abandoned
14
Medulloblastoma 20% of pediatric brain tumors
40% of posterior fossa tumors
15
Medulloblastoma Symptoms often those of hydrocephalus/raised ICP--headache, vomiting, diplopia Falling, incoordination Signs: papilledema, EOM paresis, ataxia
16
CP
17
CP
18
Role of Surgery First step in treatment is an operation
Establish diagnosis Open CSF pathways Many, but not all, large studies show increased survival with radical tumor resection
19
Surgical Approach Begin operation with goal of GTR
Brainstem invasion may prevent reaching this goal Do not chase tumor into brainstem; leave the “carpet” of tumor on floor of IV ventricle
20
Role of Experience Experience of surgeon does play a role in degree of tumor resected This may effect outcome
21
Extent of Resection by Type of Neurosurgeon
________________________________ General (76%) Pediatric (91%) X2, p ‹0.02
22
Role of Irradiation Effective Dose to posterior fossa › 50 Gy
23
Outcome Radiation Dose Relapse-free survival (%)
50 Gy (15 pt) Relapse-free survival (%) P<0.01 <50 Gy (43 pt) Years (no.) CP
24
Effects of Radiation Many studies show inverse relationship between age at irradiation and intellectual outcome Current trend is to decrease dose of radiation and add chemotherapy
25
Neuraxis Irradiation A CCSG study suggested early failure in patients given chemotherapy and 27 Gy vs 36 Gy Late follow shows curves converging SIOP study shows no difference
26
Role of Chemotherapy Studies from CCSG, POG, and SIOP all demonstrate increased survival in high-risk patients treated with adjuvant chemotherapy Active drugs include platinum, ENU’s, cytoxan, etc.
27
Role of Chemotherapy In a single institution study, the use of a three-drug regimen in high-risk patients resulted in a better overall survival than in the normal-risk patients treated with irradiation alone Recent European trial of chemotherapy alone in patients less than 3 years shows high survival
28
Outcome Radiation + Chemotherapy
Study group Historical Probability Probability Study group Historical Months post on study Months post on study CP
29
Prognostic Factors Age ‹2 years, poor prognosis
CSF dissemination, poor prognosis Radical resection, good prognosis
30
CSF Dissemination
31
Histologic Markers Tumors can be divided into classical and anaplastic tumors. Patients with anaplastic tumors do worse Patients with large cell variant do worse
32
Laboratory Studies Growth factors and receptors may be important in medulloblastoma growth IGFR-1 trk/neurotrophins
33
Laboratory Studies Sonic Hedgehog pathway important in at least some tumors Wnt pathway important in at least some tumors Notch2 may be important for growth, Notch1 may inhibit growth, of most tumors Simply says developmental pathways may be important
34
Laboratory Studies Ptch pathway alterations associated with desmoplastic variant which may have an improved prognosis Increased expression of Notch pathway gene, Hes1, has been reported to have a worse prognosis in one study
35
Ependymoma 6% of pediatric brain tumors
70% occur in the posterior fossa Hallmark on imaging is extension out of the foramina of the fourth ventricle into the CPA or cervical canal
36
Medulloblastoma-Survival
Current best 5-year survival rates are 70% Not too bad! Survival tempered by cognitive deficits from irradiation Survival continues to fall after 5 years. We need radical, new treatments that are effective and eliminate use of radiation
37
Ependymoma Signs and symptoms of hydrocephalus
May be prominent vomiting from invasion of floor of fourth ventricle
38
Ependymoma
39
Ependymoma
40
Ependymoma
41
Ependymoma Treatment consists of radical resection
No question that prognosis is greatly influenced by extent of resection Patients with radiographically confirmed GTR have greater than 80% five-year survival; 20% or less for less than GTR
42
Ependymoma Role of radiation therapy is not well established, but may be efficacious Stereotactic radiation may be effective, but may fail from dissemination in the face of local control Role of chemotherapy is not established
43
Ependymoma Surgeon has a tremendous influence on progress
Every attempt should be made to resect entire tumor Removal of tumor from floor of fourth ventricle is controversial, better prognosis vs major neurologic deficit
44
Ependymoma Studies suggest small amount of residual disease does not effect prognosis Most surgeons do not chase tumor into the floor of the fourth ventricle
45
Astrocytoma Symptoms and signs depend on location. Posterior fossa-symptoms and signs for hydrocephalus. Cerebral hemispheres-focal deficit, seizures. Tumor behavior depends on histology Tumor treatment depends on histology and location
46
Cerebellar Astrocytoma
Pilocytic astrocytoma Diffuse, grade II astrocytoma Grade III or IV astrocytoma in the cerebellum is rare in children
47
Pilocytic Astrocytoma
48
Pilocytic Astrocytoma
49
Pilocytic Astrocytoma
Surgical disease We try to remove all tumor But we do not chase tumor into cerebellar peduncle, brainstem
50
Pilocytic Astrocytoma
Post op scan clean, follow 6 month scan clean, may not need any further studies
51
Cerebellar Astrocytoma
Treatment for pilocytic astrocytoma is resection. A gross total resection is goal Controversy: Immediate reoperation for residual tumor?
52
Cerebellar Astrocytoma
53
Cerebellar Astrocytoma
Patients with grade II tumors and GTR do as well as patients with pilocytic tumors and GTR Role of radiation and chemotherapy for residual tumor in brainstem is unresolved
54
Cerebellar Astrocytoma
Diffuse, grade II astrocytoma Outcome relates to degree of resection Try for GTR Brainstem invasion prevents this
55
Cerebellar Astrocytoma
56
Cerebellar Astrocytoma
57
Cerebellar Astrocytoma
If postoperative scan shows residual tumor, only about 1/3 will show growth over next 10 years Reasonable to follow for symptoms and with scans, reoperate for progression
58
Brainstem Tumors Called “brainstem glioma”, but not all tumors in the brainstem are the same. Symptoms and signs of brainstem dysfunction: diplopia, swallowing problems, facial weakness, long track signs
59
Focal Brainstem Astrocytoma
Usually in midbrain, medulla, or cervicomedullary Dorsally exophytic into IV ventricle Account for 30%
60
Focal Brainstem Astrocytoma
Pathology: grade I, grade II astrocytoma, ganglioglioma Less often grade III or IV astrocytoma
61
Focal Brainstem Astrocytoma
Controversy about treatment Role of resection Role of radiation Role of chemotherapy
62
Focal Brainstem Astrocytoma
Subtotal resection may be effective Recommended for dorsally exophytic tumors
63
Focal Brainstem Astrocytoma
Role of surgery has yet to be defined Radical resection is a tour de force, but is it needed? Many reports of tumors that have remained stable for years with no treatment
64
Treatment of Residual Tumor
Controversy about role of chemotherapy No randomized prospective study No good study with adequate follow-up for this slow-growing tumor
65
Focal Brainstem Astrocytoma
Radical resection is possible, but at a cost True incidence of complications is not known Reported at least 50%with tracheostomy and gastrostomy Is it worth it?
66
Focal Brainstem Astrocytoma
No rush to treat. Many are slow growing and cause few symptoms Stereotactic biopsy may be used to establish histology, if needed Treatment for documented growth and/or symptom progression
67
Diffuse Pontine Astrocytomas
Characteristic image, diffuse infiltration of the pons Unresectable No role for biopsy, as patients do poorly regardless of histology
68
Diffuse Pontine Astrocytoma
69
Diffuse Pontine Astrocytoma
This is the tumor referred to as “brainstem glioma” Account for 70% of brainstem tumors Do not call others (focal brainstem tumors) “brainstem gliomas” because the prognosis is so different
70
Diffuse Pontine Astrocytomas
No controversy, 2 year survival is less than 5%. What is needed is radical, new, effective therapy
71
Diffuse Pontine Astrocytomas
No effective treatment Conventional or hyperfractionated radiation are palliative No effective chemotherapy
72
Conclusions Brain tumors in children are not common, but must be kept in mind for the child with headache or neurologic symptoms or signs. Subtle findings may be important. “One perceives only what one actively seeks.”
73
Conclusions Outcome is not as bad as generally thought
Pilocytic astrocytomas, grade II astrocytomas, gangliogliomas, choroid plexus papillomas, dermoid tumors all can be treated effectively with surgery alone Medulloblastoma, ependymoma, malignant germ cell tumors have greater than 70% long term survival with surgical resection, radiation therapy and chemotherapy
74
Conclusions Irradiation is bad for the brain
Current research directed at finding focused therapy based on what is known about the molecular biology of the different tumor types.
Similar presentations
© 2025 SlidePlayer.com. Inc.
All rights reserved.