Pediatric Brain Tumors
Terms
undefined, object
copy deck
-
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 resectionâ€), 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 “chemotherapyâ€.
- 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 knifeâ€
- ________ ___________ is a very precise form of radiotherapy; it is less useful if tumor margins indistinct or mets are likely
- Stereotactic radiosurgery (aka “gamma knifeâ€
- _______ 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)