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What are the important factors to look at regarding pediatric brain tumors
- - age
- - location of tumor
- - histology important (what kind of tumor)
- - extent of surgical resection
- delay in diagnosis does not impact survival (biology of tumor is more important than delay)
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Importance of age
infants-- nonspecific, macrocephaly, diencephalic syndrom (thin skin, motor hyperactivity, abnormally alert, failure to thrive)
Young kids--nausea, morning emesis (vomiting), gait abnormality, visual findings
Older kids--localizing symptoms, ocular manifestations, visual changes, precocious puberty, seizures
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Epidemiology
- neonate--supratentorial (division of the brain into two parts, the upper part) ---teratomas that start at conception and grow with the baby
- <2 yrs--supratentorial (astrocytoma, medulloblastomas. teratomas)
- 2-12 yrs--infratentorial (lower part), e.g. brainstem glioma
- >12 years --supratentorial
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General Management methods
Surgery-- complete reserction is the goal
Radiotherapy--growth retardation, endocrine dysfunction, progressive decrease in QU, risk of 2nd degree tumor)--not done in kids under 3-- DELAYED AS LONG AS POSS
Chemotherapy--used to delay radiation therapy because of side effects
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Radiotherapy
-acute side effects-- fatigue, anorexia, exacerbation of edema
-early delayed effects--Lhermitte's sign (electrical feeling that goes down the back), and somnolence syndrome (lethargy, drowsiness after radiation)
- Late delayed effects00neurocognitive deficits, endocrine dysfunction, radiation necrosis (death of healthy cells), hearing loss
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Hydrocephalus treatments
- - relieve pressure with steroids and surgical resection to restore normal CSF pathways
- - tube put in to drain pressure
- - ventriculocisternostomy-- opening is created that allows cerebrospinal fluid to drain through a shunt from the ventricles of the brain into the cisterna magna.
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Medulloblastoma (most common)
- -hydrocephalus, 25% require a shunt after surgery
- -dense, cellular pattern--highly cellular diffuse masses made of small, undifferentiated oval or cound cells -rosette patterns --tumor cells arragned in a circle around a fibrillary center
- -doesn't have to be completely removed
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Cerebellar astrocytoma
- - grade 1 tumor
- - take it all out, 90% of time it is cred
- - if some is left, wait to see if it grows, if it does, try to remove it or use radiation (only used with
- recurrence or unresectable tumor)
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Brainstem glioma
subtypes
- - 4 subtypes
- - diffuse--most common, can't be removed
- - focal (tectal)--increased recognition with MRI, can be removed
- - dorsally exophytic--growing outward
- - cervicomedullary
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Brainstem giloma-- surgery
- - focal--if it grows, may suggest a more aggressive tumor, and may benefit from surgery
- - dorsally exophytic--remove exophyticp ortion without entering brainstem
- - cervicomedullary--radical excision early prior to neuro deterioration
- radiation for diffuse tumors, with focal, just do observations
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Ependymoma
- - not chemo or radiotherapy sensitive
- - grade 2 turmors, should be easy to treat, but they come back
- Treatment: steroids plus early surgery
- - complete resection possible in 1/3 to 1/2
- - radiation theraphy prolongs survival
- - chemo used to delay radiation
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Ependymoma--outcomes
- 50% have a 5 year survival
- factors: extent of resection, age, histopathology, location--spinal > supratentorial> infratentorial
- recurrence-- usually at primary site, do survellience
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Ependymoma--cells
tumor cells resemble normal ependymoma cells and are arranged in perivascular formations, tubular structures and papillary formations , like to form circles around things
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Craniopharyngioma
- - benine
- - most common non glial intracellular mass in pediatric kids
- - affects kids 5-15 and adults 50-60
- - presentation---> increased intracranial pressure, headaches, visual changes, endocrine disturbance such as growth failure, diabetes insipidus, hypothyroid, sexual/menstrual dysfunction
- - large tumors can cause psychiatric changes
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Craniopharyngioma--treatment
- - surgery-- total resection only in 50%,
- - extent of resection guided by hypothalamic involvement, location of optic chiasm
- - major hormone replacement in 85%
- - radical resections, biopsyies, radiation
- - 70% recur within 3 years
- - visual recovery best with aggressive removal
- - permanent diabetes insipidus-- 80% after resection
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Pineal tumors
- - aqueductal compression and hydrocephalus
- - Parinauds (abnormalities of eye movement and pupil dysfunction)
- - precocious puberty
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Pineal tumors--treatments
- - hydrocephalus--endoscopty and biopsy
- - surgery
- - goals are diagnosis, tumor debulking (removing as much as possible), opening CFS pathways
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causes of Pediatric brain tumors
- - sporadic
- - syndromic
- - radiation exposure
- between 2 and 12 majority of tumors in posterior fossa
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Adult brain tumors
- primary brain tumors are slightly more common than metastatic
- - GBM most common (primary no p53 mutation and secondary a p53 mutation) (also pediatric GMB but
- less common)
- - Meningioma
- - Pituitary tumors
- - vestibular schwannomas
- - posterior fossa tumors in adults are most likely metastatic
- - metastatic tumors 50% of adult cancers
- - adults, 2/3 of tumors likely to be supertentorial in cerebrum
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Astrocytoma
- - grade 4, most frequent primary brain tumor
- - peak age is 45-60,
- - median survival 8-18 months
- with surgery--4-6 months
- with steroids-- 4 weeks
- with sugery and radiation-- 9 months
- with surgery and radiation and chemo--10-12 months
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Meningioma
- - benign
- - slowly enlarging, rarely infiltative
- - risk factors-- rdiation, delectio of chromosome 22
- - more common in females than males, and 3:2 brain 10:1 in spine
- - affects receptors for estrogen and progesterone and may grow with pregnancy and breast cancer
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Meningioma treatment
- - observation to watch growth, show little to no growth
- - if there is weakness in body, enlargement, or edema--indication for surgical consideration
- - surgery can cure but it depends on grade of tumor
- - not the extent of resection that is important but the histological grade of the tumor
- radiation done on residual tumor and recurrent tumors, when surgical treament is not possible because of location or medical status of the person--also radiation has a moderate effect but there are radiation complications
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Posterior Fossa tumors in adults
- - metastases
- - hemangioblastomas--most fommon in adult posterior fossa
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Neuro oncology
- brain tumros are the most common solid organ tumors in kids, second most common cancer after leukemia
- tumor histology vaires by age
- - presentation varies by age of patient and location of tumor, affects recovery after surger
- - location dictates how aggressive you can be with surgery
- - histology is the most important predictor for surgigal
- - extent of resection dictated by location of tumor--complete resection is the goal
- - radiation and chemotherapy important adjuvants for therapy
- - delayed treatment is not the most important thing, histology is
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