Tumours of the Nervous System Flashcards

1
Q

What’s the most common extra-axial tumour?

A

Meningiomas

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2
Q

What are the features of meningiomas?

A

Usually benign = arise from residual mesenchymal cells in the meninges and produce neurological symptoms by compressing the underlying brain

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3
Q

What are some examples of extra-axial tumours?

A

Meningiomas, pituitary adenomas, craniopharyngiomas, choroid plexus papilloma, acoustic neuroma

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4
Q

What is the epidemiology of nervous system tumours?

A

Primary tumours are second most common in children

Account for 2% of cancers diagnosed but commonest cause of cancer death <40

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5
Q

What is the presentation of nervous system tumours?

A

Progressive neurological deficit = 68%
Headache = 54%
Usually motor weakness = 45%
Seizures = 26%

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6
Q

What is the underlying reason for increased intracranial pressure?

A

Contribution of mass within rigid closed box (skull)

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7
Q

What are some causes of increased intracranial pressure?

A

Tumour mass, oedema mass effect, blockage of CSF or haemorrhage

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8
Q

What is the presentation of increased intracranial pressure?

A

Headaches, vomiting, mental changes and seizures

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9
Q

What are some features of tumour headaches?

A

May occur with or without raised ICP
Worse in morning = red flag if it wakes them up
Worse with coughing or leaning forward
May be associated or worse with vomiting

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10
Q

What may a tumour headache mimic?

A

Tension headache or migraine

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11
Q

What are some causes of tumour headaches?

A

Raised ICP
Invasion/compression of dura, BV or periosteum
Secondary to diplopia or difficulty focusing
Extreme hypertension
Psychogenic

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12
Q

What investigations can be done for tumours?

A

CT, MRI, lumbar puncture, PET scan, lesion biopsy, EEG, evoked potentials, angiograms, radionucleotide studies

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13
Q

What cell types are found in neuroepithelial tissue?

A

Astrocytes, oligodendroglial cells, ependymal cells, neuronal cells, pineal cells, embryonic

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14
Q

What is the most common origin of neuroepithelial tumours?

A

Astrocytes = account for 60% of tumours, 2/3 or which are high grade

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15
Q

What cell types do glial tumours arise from?

A

Astrocytes or olidodendrocytes

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16
Q

What is the grading of astrocytic tumours?

A
I = pilocytic, pleomorphic xanthoastrocytoma, subependymal giant cell
II = low grade astrocytoma
III = anaplastic astrocytoma 
IV = glioblastoma multiforme
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17
Q

What indicates the presence of a glioblastoma multiforme?

A

The presence of necrosis

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18
Q

How do low grade astroglial tumours grow?

A

Slowly = have malignant potential

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19
Q

What are some features of grade I astrocytomas?

A

Truly benign = slow growing
Usually children and young adults
Surgery is first line and curative

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20
Q

Where do pilocytic astrocytomas occur?

A

In the optic nerve, cerebellum or brainstem

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21
Q

What are the types of low grade astrocytomas?

A

Fibrillary, gemistocytic and protoplasmic

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22
Q

What are the features of low grade astrocytomas?

A

Hypercellularity, gemistocytic, vascular proliferation and necrosis

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23
Q

Where do low grade astrocytomas commonly occur?

A

Temporal, posterior frontal and anterior parietal lobes

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24
Q

How do low grade astrocytomas present?

A

Seizures

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25
Q

What are poor prognostic features for low grade astrocytomas?

A

Age > 50, focal deficit, short symptom duration, raised ICP, altered consciousness, enhancement on contrast studies

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26
Q

What is the treatment for grade II astrocytomas?

A

Surgery +/- radiation or chemotherapy

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27
Q

What is brachytherapy?

A

Radioactive isotopes applied directly = tumour receives highest dose and surrounding tissue is spared, largely replaced by radiosurgery

28
Q

What are the different types of radiosurgery?

A

High energy photons produced by linear accelerators
The gamma knife
Charged particles (less common)

29
Q

What is radiosurgery best for used for treating?

A

Highly focal malignant gliomas

30
Q

When is surgery used to treat grade II astrocytomas?

A

To control seizures or treat herniations and CSF obstruction (cytoreduction may also be used)

31
Q

What are poor prognostic indicators for grade II astrocytomas?

A

Age >45, low performance score, tumour >6cm or crossing midline, incomplete resection

32
Q

What are the two kinds of malignant astrocytomas?

A

Anaplastic astrocytoma and glioblastoma multiforme

33
Q

What are some features of anaplastic astrocytomas?

A

Can arise de novo

Median survival is 2 years

34
Q

What are some features of glioblastoma multiforme?

A

Most common primary tumour
Median survival is <1 year
Spread via white matter tracking or CSF pathways

35
Q

How are malignant astrocytomas treated?

A

Surgery = non-curative, cytoreduction to reduce mass effect, supramarginal resection if non-eloquent
Post operative radiotherapy = external beam radiation

36
Q

What are some features of oligodendroglial tumours?

A

Account for 20% of glial tumours
Adults aged 25-45 with smaller peak age 6-12
Occur in frontal lobe and present with seizures

37
Q

Where do oligodendroglial tumours infiltrate?

A

Subarachnoid space and leptomeninges

38
Q

What is the appearance of oligodendroglial tumours?

A

Solid and grey/pink cut surface

39
Q

What are collision tumours?

A

Oligodendroglial cells coexist with astrocytic cells

Originate from O2A cells

40
Q

How are oligodendroglial tumours different from astrocytomas?

A

Show calcification, cysts and peritumoural haemorrhage

41
Q

How are oligodendroglial tumours treated?

A

Surgery and chemotherapy

Median survival is 10 years

42
Q

What chemotherapy agents are oligodendroglial tumours sensitive to?

A

Procarbazine, lomustine and vincristine

43
Q

What cells do meningiomas arise from?

A

Arachnoid cap cells = represent 20% of intracranial neoplasms

44
Q

What are some features of meningiomas?

A

Mostly asymptomatic
More common in men
Histologically benign and slow growing

45
Q

What are the symptoms of meningiomas?

A

Headaches, cranial nerve neuropathies, regional anatomical disturbances

46
Q

What is a meningioma en plaque?

A

Subgroup defined by presence of carpet or sheet like lesion that infiltrates the dura = remove all bone to prevent recurrence

47
Q

What are the classifications of meningiomas?

A

Classic, angioblastic, atypical

Malignant = clear cell, chorioid, rhabdoid papillary, radiation induced

48
Q

What does a CT of a meningioma show?

A

Homogenous, densely enhancing, oedema and hyperostosis

49
Q

What does an MRI of a meningioma show?

A

Dural tail and patency of the dural sinuses

50
Q

How are meningiomas treated?

A

Surgery and radiotherapy

5 year survival is 90%

51
Q

What are some examples of nerve sheath tumours?

A

Schwannomas (neuromas), neurofibromas, malignant peripheral nerve sheath tumours

52
Q

What are acoustic neuromas?

A

Vestibular schwannomas = associated with neurofibromatosis II

53
Q

What are the symptoms of an acoustic neuroma?

A

Hearing loss, tinnitus and dysequilibrium

Hydrocephalus may occur

54
Q

How are acoustic neuromas treated?

A

Radiation and surgery
25% managed with a hearing aid
50% managed surgically

55
Q

How is the gamma knife used to treat acoustic neuromas?

A

Given to 25% of patients = usually if tumour <3cm, given in one sitting

56
Q

What are the post-op complications of acoustic neuromas?

A

Facial nerve palsy, corneal reflex, nystagus and abnormal eye movements

57
Q

What is the prevalence of germ cell tumours?

A

90% affect those <20
Peak incidence between age 10-12
More common in males

58
Q

What are some features of germ cell tumours?

A

Iso/hyperdense on CT
Often mixed histology
May metastasise via CSF

59
Q

What are the most common type of CNS germ cell tumour?

A

Germinomas = radiosensitive with 65-95% 5 year survival

60
Q

What are the types of non-germinomatous germ cell tumours?

A

Teratoma, yolk sac tumour, chroriocarcinoma and embryonal carcinoma = less radiosensitive with 17-38% 5 year survival rate

61
Q

What investigations should be done for any child with a midline brain tumour?

A

Perform AFP, beta HCG and LDH = if negative do biopsy

62
Q

How do pituitary tumours present?

A

Bitemporal hemianopia and endocrine abnormality

63
Q

How can acromegaly cause death?

A

Due to HOCM = need surgery and somatostatin analogues

64
Q

How does panhypopituitarism present?

A

Pallor, yellow tinge and fine wrinkling of skin, absent axillary hair, puffy expressionless face

65
Q

In what order are pituitary hormones disturbed in hypopituitarism?

A

GH - LH and FSH - TSH - ACTH - prolactin