NeuroOncology lecture

• - 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)

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

- 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

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

-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

• - 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.

• -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

• - 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)

• - 4 subtypes
• - diffuse--most common, can't be removed
• - focal (tectal)--increased recognition with MRI, can be removed
• - dorsally exophytic--growing outward
• - cervicomedullary

• - 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

• - 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

• 50% have a 5 year survival
• factors: extent of resection, age, histopathology, location--spinal > supratentorial> infratentorial
• recurrence-- usually at primary site, do survellience

tumor cells resemble normal ependymoma cells and are arranged in perivascular formations, tubular structures and papillary formations , like to form circles around things

• - 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

• - 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

• - aqueductal compression and hydrocephalus
• - Parinauds (abnormalities of eye movement and pupil dysfunction)
• - precocious puberty

• - hydrocephalus--endoscopty and biopsy
• - surgery
• - goals are diagnosis, tumor debulking (removing as much as possible), opening CFS pathways

• - sporadic
• - syndromic
• - radiation exposure

- between 2 and 12 majority of tumors in posterior fossa

• 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

• - 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

• - 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

• - 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

• - metastases
• - hemangioblastomas--most fommon in adult posterior fossa

- 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