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neuro 210 finallll > Neurooncology > Flashcards

Neurooncology Flashcards

1
Q

what is the manifestation of brain tumours in men vs woman?

A

in men its more malignant and they are less protected against the bad types of brain tumours but in females its more benign but happens in more females than males in total

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2
Q

What are two ‘busted’ reasons why a child can get brain tumours?

A
  1. cell phone use
  2. radiation from lab equipment which shows that dying from cancer is 23% but dying from radiation exposure is 0.35% … the risk is too low
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3
Q

What are neurocutaneous disorders?

A

they are skin problems associated with brain problems

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4
Q

what is neurofibromatosis 1 or neurofibromas?

A

its the most common neurocutaneous disorder where tumours near the nerves from ears to hearing centres in the brain and other inter cranial tumours are present (axillary or inguinal freckling)
- there can also be a optic nerve glioma .. tumour in the optic nerve

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5
Q

What are some treatments for neurofibromas?

A

neurobiromin is a tumor supressor protein … theres low need for surgical intervention

most of these patients have genetic predisposition and have benign tumours that respond nicely to chemotherapy

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6
Q

What are bilateral vestibular schwannomas?

A

tumors in the vestibular (inner ear/balance) areas … also called meningiomas and epndymomas

they can cause hearing loss, bad balance and ataxia

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7
Q

What percentage of genetic syndromes counts for paediatric brain tumours?

A

genetic syndromes account for only 5% of the central nervous system tumours the majority of the brain tumours have no underlying cause or predisposing cause

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8
Q

what is the prevalence rate of paediatric brain tumours and epidemiology?

A
  • 20% of paediatric tumours (1 in 5 kids have solid tumours)
  • most common solid tumours in children
  • second to lukeimia
  • 200 new cases diagnosed each year in canada
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9
Q

How does age determine the location of tumour growth in the brain?

A

neonates –> supratenrotial area (cortex)
birth to 2 years –> supratentorial area
2-12 years (paediatric) –> infratentorial area (brain stem)
more than 12 years –> supratenrotial area

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10
Q

when does the highest rate of tumours begin in kids? what are the majority of the tumour types that develop?

A

highest rate of tumours in kids occurs at 2-8 years of age and majority are tumours of meduloblastomas and astrocytomas

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11
Q

how do tumours present in neonates (new borns)?

A

poor apgar scores

  • failure to thrive
  • intracranial hemorrhage
  • seizures
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12
Q

How do tumours present in infants (1-2 years)?

A
  • non specific
  • macrocephaly –> head grows quickly and tumour takes up space and since their skull and brain is growing it grows around the tumour and increases ICP
  • diencephalic syndrome (thin skin, motor hyperactivity, abnormally alert…etc)
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13
Q

How do brain tumours present in toddlers (ages 2+)

A
  • headaches (due to increase ICP)
  • nausea, particularly in the mornings
  • gait abnormality
  • visual findings
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14
Q

How do brain tumours present in adolescent and adult?

A
  • lots of headaches
  • localizing symptoms (depends on the lobe)
  • ocular manifestations and visual changes
  • precocious puberty
  • seizure (15%) because the brain is irritated
  • tumours often present in lobes of supratentorium and weakness and numbers are especially if tumour is in parietal lobes
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15
Q

What three ways to manage tumours (treatment wise)?

A
  1. surgery –> complete removal of the tumour is the goal
  2. radiotherapy –> causes a lot of problems like growth retardation, endocrine dysfunction, decrease in IQ, and risk of secondary tumour… it should be avoided in children under 3 years of age
  3. chemotherapy –> use to delay radiotherapy
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16
Q

What are medulloblastomas?

A

most common paediatric brain tumour

  • its 20% of CNS tumours and 30% of posterior fossa tumours (found in the small space in skull found near the brainstem and cerebellum)
  • occurs mainly in that cerebellum area and it affects walking, balance, fine and motor coordination etc…
  • it is a tumour of primitive undeveloped cells in the cerebellum
  • causes hydrocephalus
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17
Q

what is the chang classification?

A

puts patients at standard risk or high risk depending on their age and type of tumour

standard risk patients are ones over the age of 3 with a residual tumour thats less than 1.5 cubic cm, with no metastasis (no movement)

high risk patients are ones under the age of 3 with residual tumours or metasteses

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18
Q

What are the four distinct molecular variants of medulloblastoma?

A

WNT, SHH, Group C, and Group D

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19
Q

What are subtype 1 WNT tumours of medulloblastomas?

A
  • this is a classic medulloblastoma
  • rarely have large cell or anapaestic features
  • primarily observed in older children, adolescence and adults
  • less common but has the BEST OUTCOMES

makes up the large portion of the standard risk group and does not metastasize

20
Q

what are subtype 2, SHH tumours of medulloblastomas?

A

they are sonic hedgehog tumours which include 9q deletions

  • they have a desmoplastic/nodular histology
  • they are bimodal in age distribution because it occurs in children younger than 3 years as well as adolescence and adults
  • they have a relatively favourable outcome but negatively affected by other molecular genetic changes as well

infants are more at risk

21
Q

What is subtype 3- Group 3 tumours of medulloblastomas?

A
  • characteristically MYC amplification
  • either classic or large cell/anaplastic histology
  • they are mainly metastatic (move to other parts and spread) at the time of diagnosis
  • they occur throughout childhood with a 2:1 increase in males to females
  • if you have MYC+ at age less than 4 years you have a less than 50% survival in 5-year span
  • if you have MYC- at age more than 4 years you have 70% survival in 5 years of time
22
Q

What is subtype 4 - group 4 tumours of medulloblastomas?

A

CDK6 and MYCN amplification … similar to group 3

  • either classic or large cell/anaplastic histology
  • metastases at presentation is common
  • occurs throughout childhood and into adulthood
  • prognosis is better than group 3 but not as good as WNT tumours
23
Q

What do we know about the 4 subtypes of medulloblastomas?

A
  1. WNT: less common but 95% survival regardless of extent of surgical resection (because most of these are benign)
  2. SHH - more common in infants and adults, good response to chemo, extent of surgical resection is very important because it can grow back if not removed fully
  3. type 3/group 3 - more common in adolescence, does poorly regardless of extent of resection
  4. type 4/group 4 - more common in teenagers, extent of resection does not reduce recurrence, but still surgically amenable (better than type 3)
24
Q

Why are pilocytic Astrocytomas?

A

occurs in 20% of paediatric CNS tumours and 30% of p-fossa tumours

  • these are somewhat benign
  • its a glial tumour and occurs in the cerebellum mostly
  • 70% is present in children and most of them who get it are more than 7 years of age
  • radiotherapy (XRT) is not recommended unless recurrence or unresectable tumour
  • 90% have long term survival with GTR (gross total resection)
  • management of residual tumours is done through observation, radiotherapy or SRS for growth/recurrence…
25
Q

What is a brain stem glioma?

A

occurs in 20% of paediatric CNS tumours, and 30% of p-fossa tumours

  • theres 4 distinct subtypes of it:
    1. diffuse (pontine) - 60-80%
    2. focal (tectal) - increased recognition with MRI
    3. dorsally exophytic - 20%
    4. cervicomedullary
26
Q

What are diffuse/pontine gliomas?

A

infiltrates the entire pons and theres no goal for surgery … its only treated with XRT and chemo but theres no positive outcome… it leads to death most times

27
Q

What are tectal/focal gliomas?

A

these are more of a benign kind of gliomas

  • they cover the third ventricle and push CSF back into the brain and causes hydrocephalus
  • all thats needed to do is to put a hole through the third ventricle so CSF can flow out… thats all thats needed
28
Q

What are three types of gliomas that need surgery?

A
  1. focal tumours like tectal glioma, … third ventriculostomy and observation (to get rid of hydrocephalus)
  2. dorsally exophytic –> remove exophytic portion without entering brainstem
  3. cervicomedullary –> radical excision early, prior to neurological deterioration
29
Q

what kind of glioma tumours don’t need surgical treatment?

A

Diffuse tumours because they occur in the pons and usually a radiotherapy is used to treat it and it has a 2 year survival chance of 10-20%

30
Q

What are ependymoma’s?

A

they are malignant cells that form in the tissues of the brain and spinal cord

it occurs to 10% of p-fossa tumours in children and 30% in children less than 3 years of age

  • 50% is present in children less than 5 years of age
  • the chance for a 5 year survival is approx 50%
31
Q

what are factors that affect survival in children with ependymomas?

A
  1. extent of resection
  2. age
  3. craniospinal dissemination
  4. histopathology
  5. location -> spinal > supratentorial > infratenotorial
32
Q

what are some therapy methods that can be applied to ependymomas?

A
  • its locally aggressive and unresponsive to chemotherapy because the tumours are slow growing and relentless (hard to disrupt the cell’s growth cycle when its slow)
  • PF-A is more likely in males, younger patients, has higher grade, more invasive and chemo resistant and more likely to recur with higher mortality
  • PF-B is less invasive of the floor of the brainstem and its easier to resect
  • Gross total resection still important for overall survival in PF-A. Extent of resection and radiation does not impact overall survival for PF-B
33
Q

what are suprasellar tumours?

A
  • craniopharyngioma’s
  • germinoma
  • astrocytoma
34
Q

what are pineal region tumours?

A
  • germinoma
  • non-germinomatous germ cell tumours
  • pineocytomas
35
Q

What are primary brain tumours in adults? what kind of tumours do they include?

A

primary brain tumours are somewhat more common than metastatic tumours but varies with age …

primary brain tumours occur in the brain without metates

  • they include:
    1. GBM tumors (glioblastoma multiforme)
    2. meningiomas (not a glial tumor)
    3. pituitary tumors
    4. vestibular schwannomas (not a glial tumor)
36
Q

what is glioblastoma multiforme?

A

its the most frequent primary brain tumour in adults

  • it is similar to astrocytoma
  • peak age is 45-60 in mostly males
  • median survival is 8-18 months
  • glial tumours are the largest and most aggressive
37
Q

what are 4 treatment methods for glioblastoma multiforme and what do they depend on?

A
  1. steroids –> given to usually ill patients who have a shorter survival time … this just keeps their symptoms at ease (lasts 4wk for recovery)
  2. surgery –> 4-6 month recovery
  3. surgery + radiotherapy –> 9 months recovery
  4. surgery + radiotherapy + chemo –> usually given to people who were not ill at the time of the tumour and have a higher survival rate … which extends the treatment recovery to 10-12 months
38
Q

What is a meningioma?

A

its a tumour in the surface of brain within the dura meninges

  • it is slow to form and rarely infiltrative
  • consists of 15% of primary intracranial tumours
  • peak age is 40-60 years old
  • risk factors: radiation, chromosome deletion 22, NF2
  • more in females than males with a 3:2 in brain and 10:1 in spine
  • hormone receptors for estrogen and progesterone that may rise with pregnancy and breast cancer
  • best method of treatment is to watch them to see if it will grow or show any side effects before intervention
39
Q

what 3 things need to available in order to surgically intervene with the growth of a meningioma?

A
  1. symptomatic (showing symptoms related to the tumour)
  2. enlargement
  3. edema (swelling)
40
Q

What is a Schwannoma?

A

tumour in the vestibular and p-fossa areas of the cerebellum

  • presents with:
    1. unilateral hearing loss
    2. vertigo
    3. tinnitus (ringing in ears)
    4. headaches
    5. facial numbness
    6. facial weakness (because it puts pressure on cranial nerves for the face

can cause hydrocephalus

  • treated through observation and growth
  • surgery and XRT are not as affective

loss of hearing and bad balance still ongoing even after surgery

41
Q

what type of adult tumour is likely to be metastatic?

A

posterior fossa tumors in the cerebellum
- its good to search for primary site of the tumour (where it moved from) and determine status of primary disease

these are often hemorrhagic… 4 common areas that it could arise from

  1. choriocarcinoma
  2. thyroid
  3. melanoma
  4. renal
42
Q

What are the method of radiation therapy?

A

its used mainly in rapidly dividing cells

  • it breaks double stranded nuclear DNA
  • impact on cell senescence and apoptosis
  • utilizes the biological activity of tumour cells vs. normal tissue
43
Q

What are 3 methods of therapy used to treat tumours and how specific are they?

A
  1. radiation therapy
  2. definitive, palliative, adjuvant
  3. photons vs. protons –> proton radiotherapy is focused on the tumour and side effects of other neurons being affected is less
44
Q

what are some acute effects of irradiation?

A
  1. fatigue, anorexia
  2. exacerbation of edema
  3. erythema
  4. loss of taste
  5. reduced, thickened saliva affecting swelling, taste and dental caries
  6. hair loss
  • can cause necrosis because radiation treatment sclerotized blood vessels (causes scarring in blood vessels) and swelling from the tumour could get worse
45
Q

What is chemo therapy and what are the classes and mechanisms of actions? (5)

A
  • treating a tumour with chemicals
  • 5 classes
    1. acylating agents –> alkylation of DNA leads to cross linking and inhibition of DNA synthesis
    2. antimetabolites
    3. plant alkaloids
    4. antibiotics –> block synthesis of DNA and RNA
    5. hormones
46
Q

what are the side effects of chemo?

A
  1. nausea, vomitting
  2. alopecia (hair loss and blading)
  3. bone marrow suppression
  4. neurotoxicity
  5. hearing loss (cisplatinum)

neuro 210 finallll (12 decks)

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