NeuroOncology lecture

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  1. 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)
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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.
  7. 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
  8. 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)
  9. Brainstem glioma
    • - 4 subtypes
    • - diffuse--most common, can't be removed
    • - focal (tectal)--increased recognition with MRI, can be removed
    • - dorsally exophytic--growing outward
    • - cervicomedullary
  10. 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
  11. 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
  12. 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
  13. 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
  14. 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
  15. 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
  16. Pineal tumors
    • - aqueductal compression and hydrocephalus
    • - Parinauds (abnormalities of eye movement and pupil dysfunction)
    • - precocious puberty
  17. Pineal tumors--treatments
    • - hydrocephalus--endoscopty and biopsy
    • - surgery
    • - goals are diagnosis, tumor debulking (removing as much as possible), opening CFS pathways
  18. causes of Pediatric brain tumors
    • - sporadic
    • - syndromic
    • - radiation exposure

    - between 2 and 12 majority of tumors in posterior fossa
  19. 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
  20. 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
  21. 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
  22. 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
  23. Posterior Fossa tumors in adults
    • - metastases
    • - hemangioblastomas--most fommon in adult posterior fossa
  24. 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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NeuroOncology lecture
2010-04-02 21:07:13
neuroscience oncology

neuro lecture, march 4
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