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Year 5 Pathology - Week 1 > neuroonco with steveeeee > Flashcards

neuroonco with steveeeee Flashcards

1
Q

How are CNS tumours classified?

A

Primary - if they originate in the CNS
Secondary - if they are mets (most common in adults)

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2
Q

How common are primary CNS tumours in adults and children?

A

In adults - rare (1-2 percent)
In children - most common in children under 14 years old (25 percent)

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3
Q

How do we classify CNS primary tumorus?

A

Extra axial aka coverings - tumours of bone, soft tissue, meninges, nerves

INTRA axial parenchymal - aka brain cells - glial astrocytes and oligodendrocytes, neurons and neuroendocrine cells

Lymphomas and germ cell tumours are also intra axial

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4
Q

Which CNS tumours are more malignant?

A

Extra axial - less malignant
Intra axial - more malignant since they infiltrate the tissue of the brain

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5
Q

Causes if CNS tumours

A

Unknown
Radiotherapy to head and neck
Genetic disposition (less that 5 percent)

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6
Q

Name 5 familial CNS tumour syndromes and their cancers

A

Neurofiboromatosis 1 - neurofiboroma astrocytoma
Neurofibroamatosis 2 - schwanomma, meningioma
Brain tumour polyposis syndrome 1 - malignant gliomas
Gorlin syndrome - medulloblastoma
Von Hipple Lindau - Hemangioblastoma

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7
Q

Signs and symptoms of CNS tumours

A

Space occupying lesions, therefore you get intracranial hypertension symptoms:
-Headache and vomiting
-change in mental status

If it’s above tentorium:
focal neurological deficity
Seizures
Personality changes (esp if in frontal lobe)

Infratentorial
Cerebella ataxia
Long tract signs
Cranial nerve palsy (esp oculomotor nerve if it’s pressing on it)

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8
Q

Treatment option for CNS tumours

A

Surgery - resection, but this depends on the type of tumour. Resection means that the surgeon will need to resect a part of normal tissue around the margin of the tumour.
Radiotherapy - low and high grade gliomas
metastases, bening tumours

Chemotherapy
Mainly for high-grade gliomas (temozolomide) and lymphomas

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9
Q

Where do medulloblastomas come from?

A

embryonal cells

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10
Q

WHO grading of CNS tumours

A

Grade 1 - benign long term survival
Grade 2 - more than 5 years
Grade 3 - less than 5 years
Grade 4 - less than 1 year

Note - some tumours have only one possible grade, but others have more than one grade .

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11
Q

Diffuse glial tumours

A

Diffuse gliomas - grade 2 or above
Seen in adults
Supratentorial
Malignant progressoin

Astrocytomas (g2-4)
Oligodendrocytomas (grade2-3)

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12
Q

Circumscribed gliomas

A

Seen more in children in posterior fossa, rare malignant transformation

Pilocytic astrocytoma (g1)
Subependyml giant cell astrocytoma (g1)
Ependymomas

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13
Q

Most common gliomas seen in kids under the age of 14?

A

PILOCYTIC ASTROCYTOMAs (fifth of all gliomas in kids)

BRAF gene mutation in 70 percent of pilocytic astrocytomas

well circumscribed lesion

Pilocytic = piloid hairlike cells
Rosenthal fibres seeen o nH and E
Slow growing, low mitotic activity

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14
Q

Diffuse gliomas and their key mutation

A

Astrocytoma - IDH mutant, point mutation

Oligodendroglioma - IDH mutant

IDH mutation is associated with longer term survival, point mutation

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15
Q

Astrocytoma sings

A

Low choline/creatine raio on MR spectroscopy
T1 - hypointense
T2 - hyperintense

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16
Q

Astrocytoma histology signs

A

Astrocytoma

Slow growth
No vascular proliferation
IDH1 mutation can be detected on immunocytochemistry

17
Q

What is the worst type of glioma?

A

Glioblastoma multiforme (GBM) grade 4
Supratentorial
Median survival only 8 months
Most patients over the age of 50 years

IDH1 wildtype
TERT, PTEN, EGFR mutations

MRI - heterogenous

18
Q

Histopathology of GBM

A

High cellularity. lack of clean margins
Necrosis
Neoangiogenesis

19
Q

Meningioma

A

Well circumscribed
Meningothelial cells of arachnoid matters
Grade 1 - 80 percent
Grade 2 -
Grade 3

Grading is the most useful marker of recurrence

TERT mutation
Methylome

20
Q

What do you have to check?

A

High mitotic activity - you count the number

Microscopic brain invasion -

21
Q

CNS mets are most commonly found where?

A

Grey-white matter junction
OR
Leptomeningeal ????

22
Q

Where do mets come from?

A

Lung cancer
Breast cancer
Melanoma
Renal cell carcinoma

23
Q

Medulloblastoma

A

Embryonal tumour - second most common brain malignancy in children despite being rare

Outcome improved with radiotherapy

24
Q

histological profile? medulloblastoma

A

Synpatophysin
GFAP
Ki67

High grade, poorly definitiated

25
Q

Methylome profile

A

Methylation of CpG islands can tell us about the tumour cell of origin

26
Q

What is NF1?

A

A microdeletion of NF 1 gene on chromosome 17 which results in a range of problems.

27
Q

Symptoms/signs of NF1

A

N = neurofibroma
F = freckles under armpit/groin aka axillary or inguinal
L = Lisch nodules aka spots around iris
L = cafe au Lait spots
L = light (optic) pathway glioma

(L looks like the number 1 in NF1)

28
Q

What is a neurofibroma?

A

Peripheral nerve sheath tumour that creates soft bumps under skin

29
Q

What is the problem in NF2?

A

Autosomal dominant, problem with chromosome 22

30
Q

What are the signs and symptoms of NF2?

A

Bilateral = everything is doubled aka TWO aka NF TWO

Bilateral schwannomas
Bilateral cataracts
Meningiomas
Ependymomas

31
Q

How do bilateral schwannomas manifest?

A

In bilateral hearing loss as it is also called an acoustic neuroma as it affects CN 8

32
Q

What is an ependymoma?

A

It is a tumour of the 4th ventricle, leads to raised ICP

33
Q

What is the difference between a schwannoma and a neurofibroma?

A

Neurofibroma - tumours of small nerves in ANY organ

Schwannoma - tumours of large nerves in the brain and spinal cord

Both are ‘peripheral’ nerve tumours however.

34
Q

What type of cancer is associated with NF1?

A

Neurofibroma and astrocytoma

35
Q

What type of cancer is associated with NF2?

A

Schwannoma and meningioma

36
Q

Malignant gliomas are associated with which inherited syndrome?

A

Brain tumour polyposis 1

37
Q

Gorlin syndrome - which tumours are associated?

A

Medulloblastoma
(and also naevoid basal cell carcinoma syndrome)

38
Q

Which tumour is associated with von Hippel Lindau?

A

Hemangioblastoma

39
Q

What is hemangioblastoma?

A

Cancer of the blood vessels in the brain, spinal cord or retina

(vascular tumours of the central nervous system)

Year 5 Pathology - Week 1 (26 decks)

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