Lecture 4 - Brain Tumours Flashcards Preview

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Flashcards in Lecture 4 - Brain Tumours Deck (34):
1

Why have survival rates of children with brain tumours increased?

What are some consequences of this?

* Dramatic increase in detection and treatment efficacy of brain tumours

* Greater neuropsychological, psychological and medical late effects in survivors of pediatric brain tumours

2

Paediatric brain tumours along with other CNS cancers account for __% of all childhood cancers?

20%

3

The risk peak for Paediatric Brain Tumours is between __ and __ years

3 and 9

4

What factors affect survival rates and outcomes in children with brain tumours?

Tumour type (histological); Location; and Malignancy. Age at diagnosis. Age at treatment. Type of treatment. Whether there are other complications (e.g., hydrocephalus)

5

What are some of the treatments for paediatric brain tumours?

Treatments:
–Biopsy
–Surgery (resection, debulking)
–Chemotherapy
–Radiotherapy

6

What is the difference between chemo- and radio- therapy?

Chemotherapy refers to a chemical or drug given to destroy cancer cells (it has systemic side-effects because it travels through the blood-stream).
Radiation therapy is a local high energy X-ray treatment, focusing only on the areas affected by cancer, though can also be whole-brain (There is a dose-effect relationship)



EXTRA NOTES: The chemo drug is given through an intravenous (IV) injection, it spreads throughout the entire bloodstream. This type of treatment is effective because it kills cancer cells that have spread beyond the initial affected tissue or organ. On the other hand, as the drug spreads through the bloodstream killing cancer cells, it also has effects on other rapidly dividing cells such as those in your blood, mouth, nose, nails, vagina, intestinal tract and hair. This can cause patients to feel ill after treatment and lose their hair.

7

What are some of the pathophysiological consequences of Radiotherapy treatment?

•IQ reductions (Can start a year or so after treatment)
White matter changes (though not a huge amount of research)
•Radiation induced dementia - slow and progressive cognitive decline with associated cortical atrophy that can begin months to years after treatment. brain can shrink up to 5%.
•Focal cerebral necrosis
•Cerebrovascular disease (and other malformations of blood vessels)
•Radiation induced communicating hydrocephalus

8

What are some of the long-term effects of RT?

–Neuropsychological, educational, psychosocial, behavioural
–Abnormal growth and hormone function
–Liver, kidney damage
–Respiratory problems
–Gasointestinal, cardiac problems
–Hearing/Vision Impairments
–Skeletal, spinal, skin problems
–Second cancers
–Motor problems
–Seizures

Anywhere there are maturing cells may be affected!

9

Why do doctors hold of on radiotherapy? what do they try to use in the mean-time? what age do they try to wait til?

Radiotherapy is very damaging particularly on development, chemotherapy is often used to hold off until at least the age of 6.

10

What is the main problem with both chemo and radio-therapy in treating children?

Both cause cell death, particularly in cells that are less mature. Treatments themselves can have neuropsychological outcomes

11

What is radiotherpay designed to do?

It is designed to inhibit DNA synthesis or interfere with cell division.

12

What is radiation induced dementia?

A slow and progressive cognitive decline with associated cortical atrophy that can begin months to years after radiation treatment.

13

What overall domains can brain tumours affect? (generally)

–IQ
–Attention & Concentration
–Speed of processing
–Working memory
–Language
–New learning and memory
–Academic functioning
–Executive functioning

BUT deficits are varied and are not necessarily stable

14

What is Malignancy? and what are the grades?

How much the Tumour infiltrates the cells (i.e., how easy it will be to remove)

–GRADE I, Benign
–GRADE II, Semi-Benign
–GRADE III, Semi-Malignant
–GRADE IV, Malignant

15

What is the histological type? and what are the different types?

Cell type.

Neuroepithelial (most common- a type of stem cell)
–Astrocytoma
–Medulloblastoma
–Ependymoma

Mesodermal (these compress, rather than invade surrounding tissue - with typically better outcomes)
–Meningioma
–Sarcoma

Ectodermal (maldevelopment of cell formation)
–Craniopharyngioma
–Pituitary adenoma

16

What are the classifications of tumour location? Which is more common in children?

–Infratentorial: Cerebellum, 4th ventricle, brainstem (most common in children)

– Supratentorial: Cerebral hemispheres, midline, ventricular system, thalamus & basal ganglia

17

What are Posterior Fossa Tumours? Where are they in the brain, what physical effects do they have on surrounding brain structures and what are pathophysiological consequences are associated with them?

An infratentorial tumour occuring in the posterior fossa resulting in displacement of the 4th ventrical, brainstem compression, and herniation.

Associated with hypertension increased hypertension, increased intracranial pressure due to

18

What are the three types of posterior fossa tumours discussed in class?

1.Cerebellar Astrocytoma
2.Medullablastoma
3.Ependymoma

19

What is a Cerebellar Astrocytoma? (provide details)

A relatively common Posterior Fossa Tumour type typically,
–Relatively benign, slow growing
–Cystic, rather than infiltrative
–Surgery and post-operative monitoring
–Good prognosis
-- But can have hydrocephalus as a secondary complication due to displacement of the 4th ventricle

20

What is a Medullablastoma? (provide details)

A most common (+50% of all childhood tumours) type of Posterior Fossa Tumour type typically,
–Arises from cerebellar vermis in the region of the 4thventricle
Malignant, rapid growing (survival rate 60%)
–Cellular and poorly demarcated
–Surgery and radiotherapy - though it is very difficult to remove these tumours because they are poorly demarcated from surrounding tissue.
–Increasing rates of survival
–Cognitive and behavioural sequelae - IQ typically

21

Broadly, what are some of the cognitive and behavioural sequelae of Medullablastomas?

IQ typically worse; possibly due to white matter pathology).

80% perceptual-motor impairment.

Academic impairment is common, particularly in maths.

Psychosocial defcitis and psychopathology also common (which is not a usual in tumors as it is in TBI)

22

What is a Ependymoma?

A less common (8-10% of childhood tumours) type of Posterior Fossa Tumour that arise from the 4th ventricle and penetrate the brainstem subtances, typically,
–Arise from 4thventricle
–Peak incidence first 2 years of life
–Resection (usually incomplete - brainstem involved!) + Radiotherapy (and chemotherapy for that)
–Poor prognosis - despite being relatively benign (not very infiltrative) it has a dangerous location

23

What is Cerebellar Mutism?

A phenomenon that occurs following the removal of posterior fossa tumours. Particularly were tumours involve the vermis, deep nuclei of the cerebellum and both cerebellar hemispheres.

•A syndrome of mutism and subsequent dysarthria (complete transient loss of speech that resolves into dysarthria)
•Is a 'syndrome' --> Often accompanied by personality changes, emotional lability and/or decreased initiation of voluntary movements
•The spectrum of associated neurological and behavioural anomalies varies widely among patients
•Most cases regain language, but may be residual deficits
** Speech abnormalities might include: Articulation difficulties, prolonged phoneme, slow rate of speech, hoarse voice, shotgun/explosive speech etc

24

What is a common speech issue following tumours involving the cerebellum?

Cerebellar Mutism

25

What are the three types of Midline tumours discussed in class?

1.Craniopharyngioma
2.Pineal Tumours
3.Pituitary Tumours

26

What is a Craniopharyngioma?

A type of midline tumour, typically,
–Histologically benign but semi-malignant in behaviour
–Usually slow growing but marked tendency to re-occur; often lodged behind the optic chiasm (frontal lobes)
–Peak incidence between 5 and 10 years of age
–Most often suprasellar and may damage optic chiasm or hypothalamus
–Good prognosis in terms of survival, neuropsych. abnormalities though
- Because of location, associated with neuroendocrine disorders and visual defcitis

27

What are some of the neuropsychological outcomes of Cranipharyngiomas?

Because of location (optic chiasm and hypothalamus), associated with neuroendocrine disorders and visual deficits; + neuropsych and psychosocial problems.

Generally thought intact IQ, but difficulties with memory impairment (encoding and retrieval) some of which is probably secondary to executive function (poor planning, inhibition, and problems with flexibility).

28

What is a Pineal Tumour?

A midline tumour type, typically,
–50% are germinomas (usually malignant)
–Infiltrative and rapidly growing - generally poor prognosis
–Peak incidence around puberty

29

What is a Pituitary Tumour?

A midline tumour type, typically,
–Most are adenomas ( benign tumours formed from glandular structures in epithelial tissue[lines organs and blood vessels])
–Histologically benign, slow growing

30

Are tumors of the cerebral hemispheres more common in younger or older children?

Younger

31

What are some clinical/medical manifestation of tumors of the cerebral hemispheres?

• Clinical Manifestations include raised ICP + focal neurological signs and seizures

32

What are three common types of tumours of the cerebral hemispheres?

1.Low grade Astrocytomas - generally good prognosis
2.Ependymomas - reoccurance common, poor prognosis
3. Oligodendrogliomas - rare, occur in frontal lobe, often include seizures.

33

CASE 1 - 11 year old

Is this consistent with a medulloblastoma tumour?....What Recommendations would you make?

Medulloblastomatumour resected -Following surgery he lost speech and motor skills and was later diagnosed with cerebellar mutism. Also presented with right hemiparesis, nystagmus (visual scanning, horizontal and verticle), ataxia and facial nerve palsy

Treatment
•Surgery
•RT
•CT
Speech Assessment
•4 months post-surgery-dysarthricspeech, poor breath control, mildly reduced pitch variation in speech, slow speech rate and low volume.
•Following therapy, pleasing results after 6 months though some difficulties in speech persisted.
Occupational Therapy Assessment
•6 months after resection –poor balance, coordination and
•motor planning

Background to Neuropsych. Ax.
•Repeated Year 5
•He was attending school 2 to 3 hours, three mornings a week
*Borderline IQ (reading and maths) - tested at school
•Learning support program
•He had recently ceased CT
•Approx. 1 year post RT
•Recent MRI showed no tumour recurrence
*Parents noted fatigue

Presentation
•Pale and thin, nasogastric tube for feeding
•Cheerful, but shy
•Speech –poor initiation, slow, dysarthric.
•Alert, motivated and very cooperative.

On testing.

*memory, perhaps relating to interference, and perhaps on delay
*Verbal retrieval problems - word finding and stories
*Processing speed, fatigue/language/visual scanning issues complicated the picture
*Comprehension of instructions (expressive and receptive language)

YES, cerebellar mutism and remaining speech effects (reduced pitch, variation in speech, slow speech rate, low volume).




Recommendations
•Ongoing part time schooling, monitor progress and gradually increase school attendance (not ready yet for full-time regarding fatigue and abilities)
•Ongoing educational support
•Review in 18 months, prior to starting high school

34

Case 2 - 12 year old girlCraniopharyngioma
–Craniopharyngiomatumour (left frontal lobe), resected
–Following surgery she developed diabetes, adrenal insufficiency, hypothyroidism and bitemporalhemianopia

Presentation
•Polite, mature, co-operative
•Slightly impulsive on drawing tasks
•Slightly anxious

NA