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Pediatric Brain Tumors

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Epidemiology: Ca. (40/60/80%)
of ped. pts. diagnosed b/w ages of 0-10
80%
60% of PBTs occur in the ________ fossa, vs. adults who usually get tumors that are ______tentorial
posterior; supratentorial
The posterior fossa is the “back vault” of the brain, and contains the ______ and ______.
Cerebellum and brainstem (midbrain, pons, medulla)
What is the overall 5 yr. survival rate for PBTs?
50 of children with PBTs survive 5 yrs, but there is great variability.
Name 3 factors that effect the survival rate in PBTs.
3 factors that affect prognosis are:
Age
Tumor type
Tumor location
Name three common types of brain tumors in children.
1. Low-grade astrocytomas
2. Primitive Neuroectodermal Tumors (PNETS)
3. High-grade gliomas
(Less common in children, more in adults)
Low-grade Astrocytomas: How are they commonly treated?
Frequently treated by surgery alone
Low-grade Astrocytomas: What is the prognosis?
If entire mass is resected (“gross total resection”), px is very good. In this case, 90% survive 10-20 years
Low-grade Astrocytomas: When are they more likely to be fatal?
If located in the brainstem (& therefore less likely to be "resectable") they are much more likely to be fatal
Primitive Neuroectodermal Tumors (PNETS):How malignant are they?
Often fairly malignant, therefore requiring more aggressive treatment
Primitive Neuroectodermal Tumors (PNETS): Name the most common type of PNET.
medulloblastoma
Primitive Neuroectodermal Tumors (PNETS): What is the prognosis for the tumor type mentioned in the previous card, the most common PNET (m___________a)? 5 year survival rate of __to__%.
“standard risk” medulloblastoma has 5-yr survival rate of 50-80% after surgery, chemotherapy and radiotherapy
High grade gliomas: Name a type of high grade glioma. This type is most common in (kids/adults).
Glioblastoma multiforme; adults
G________ m_________ tumors are often fatal and therefore require aggressive treatment
Gliblastoma multiforme (plural, multiformae)
Without treatment, prognosis for gliblastoma multiformae is ca:
3 months
With aggressive treatment, prognosis for gliblastoma multiformae is ca:
1-2 years, typically
What is the first step in the neurosurgical process?
Surgery is preceded by a biopsy of the tumor.
In the case of acute obstructive _____________, a _________ is placed.
A shunt or stent is placed in the event of acute obstructive hydrocephalus.
What is the relationship between percent of tumor resected and cognitive outcome?
There is no consistent relationship between % of tumor resected and NP outcome
What impact does surgery for PBTs typically have on NP outcome?
Surgery may increase risk of NP deficits, but often only slightly
While surgery may often increase risk of NP deficits only slightly, surgical _______ are assoc. with poorer outcome
complications
Name 2 common types of chemotherapeutic agents (broadly, not specific chemicals).
Antineoplastic treatments and steroids
What are an___________c treatments?
Antineoplastic treatments are what we usually think of as “chemotherapy”.
Like an_________c treatments, s_______s may have NP effects
antineoplastic treatments; steroids
Most agents administered into the _____________, but methotrexate may be administered___________ (i.e. directly into CSF)
bloodstream; intrathecally
NP effects of chemotherapy ____ by agent, some are more ____ than others
vary, benign
Possible short-term effects of Chemotherapy sequelae may include lower _____ ____ speed/_______, and ________ ability. This may be due to at least partially reversible p________ n_________
fine motor speed/coordination; graphomotor ability; peripheral neuropathy
Long-term sequelae: how does chemotherapy compare to radiation treatment?
Chemotherapy sequelae less marked than radiation, but variety of agents/combos/ delivery means variety in outcomes
Name one type of chemotherapy that is known to heighten long-term effects of radiation
Methotrexate may heighten long-term cognitive effects, moreso if administered intrathecally
Long-term steroid use may affect performance in ________ and _______(academic skills), as well as the NP abilities of __________ and _____________.
Long-term steroid use may affect reading and math, as well as immediate attention and visual/constructional and drawing skills.
Name four types of radiotherapy:
Craniospinal; Focal; Craniospinal with local boost; Stereotactic radiosurgery (aka “gamma knife”
________ ___________ is a very precise form of radiotherapy; it is less useful if tumor margins indistinct or mets are likely
Stereotactic radiosurgery (aka “gamma knife”
_______ radiotherapy minimizes exposure of healthy tissue
Focal
_______ radiotherapy targets the whole ______ ______. This targets the tumor and also helps reduce metastases.
Craniospinal; CNS axis
__________ radiotherapy with a ________ _________ both strongly targets the tumor and gives a lowered dose to the ____________ axis to reduce ____________.
Craniospinal radiotherapy with local boost; CNS; metastases
What is the effect of radiotherapy on IQ?
IQ may drop 5-7 points per year (variable though)
When does the effect of radiotherapy on IQ begin and end?
Delayed onset -- 1-2 years;
Unclear when decline stops
How does the effect of radiotherapy on IQ differ based on pt. age?
The younger the child, the more severe; not a phenomenon seen in adults (though adults may still have some NP effects, e.g. complaints of memory or attention difficulties)
How might radiotherapy sequelae be reduced?
Lowering the dose to the whole brain and using a focal boost may improve outcome
What are common NP effects of radiotherapy in children?
No single NP profile, but may affect: memory, attention, fine motor speed and coordination, math skills, visual-spatial & visual-construction skills
What are some of the mechanisms that lead to changes in cognitive functioning with radiotherapy in children? (4 factors)
White matter degeneration, poor myelination-- (partly why this a developmental issue); Vasculo-occlusive changes;
Skill stagnation adding to IQ drop; Mood factors may also have negative impact on performance
Which have greater likelihood of long-term NP impairment? Midline and infratentorial or supratentorial tumors?
Supratentorial tumors in cerebral hemispheres gen. assoc. w/ greater long-term NP impairment than midline and infratentorial tumors.
Are initial tumor size and residual volume consistently associated with NP outcome?
No, not consistently
Does recency of sx onset relate to outcome?
Recenty of sx onset may be predicted of cog. outcome; recent sx onset and rapid decline may reflect more aggressive tumors requiring more aggressive treatments
Does treated acute obstructive hydrocephalus relate to NP outcome?
No. This differs from outcome with chronic hydrocephalus.
Which types of tumors are most likely to cause seizures?
Hemispheric tumors are most likely to cause seizures.
What effect do seizures 2ary to tumors have on inter-ictal functioning?
Seizures’ efffect on inter-ictal functioning is unclear.
Do AEDs (anti-epilepsy medications) have cognitive effects?
AEDs (anti-epilepsy medications) do have varying cognitive effects.
Does treated endocrine dysfunction from cancer treatment have NP effects?
Treated endocrine dysfunction from cancer treatment doesn’t appear to have measurable NP effects.
Brain tumors are classified based on tumor site, tissue type, benign vs. malignant. Childhood nervous system tumors may be _____tentorial (located below the _____ _____) or ______tentorial.
infratentorial; tentorium cerebelli; supratentorial
The most common childhood brain tumors are ________, __________, _______, and _____ _____ _____.
astrocytoma, medulloblastoma, ependymoma, and brain stem glioma.
Cerebellar astrocytoma are usually benign, c_______. They grow (slowly/quickly).
cystic, slowly
Presenting signs of a (tumor type) usually include clumsiness of one hand, gait changes (stumbling to the left or right), vomiting, and headache.
cerebellar astrocytoma
Presenting symptoms of a __________ may include headache, vomiting, ataxia, lethargy.
medulloblastoma
Medulloblastomas may metastasize into this region:
spinal cord
Presenting symptoms for a(n) ________ include headache, vomiting and lethargy. Tumors of this type are located in the brain ventricles and obstruct the flow of CSF:
ependymoma
A (tumor type) occurs in the midbrain, pons or medulla. It occurs almost exclusively in children. Presenting signs may include double vision, facial weakness, vomiting, and difficulty in _______.
brainstem glioma; walking
(tumor type) is located near the pituitary _____. Though often benign, ______ is difficult due to its proximity to vital structures. Presenting signs include changes in _______, weight _____, headache, and change in _______ function.
craniopharyngioma; pituitary stalk; resection; vision, weight gain, endocrine function
This tumor type has a 40-90% chance of cure depending on subtype.
cerebellar astrocytoma
About 30-50% of children with (tumor type) are disease-free within 10 years. If relapse occurs it is likely to happen in the first 5 years. Children under 3 have a poorer prognosis b/c of likelier mets and lower doses of radiation.
medulloblastoma
High grade (tumor type) may be fatal, and overall childhood survival is less than 30%. If low-grade, 5-year survival rate of 80% is seen
ependymoma
Overall survival rate for (tumor type) is 20-30%.
brainstem glioma
(Treatment type) may lead to psychosis in a small proportion of individuals. This is expected to remit in the great majority of individuals after treatment completion.
Steroid administration (high dose)

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