L41 - Pathology of Intracranial Tumours Flashcards

1
Q

List causes of presenting symptoms from intracranial tumours?

A

 Focal destruction of brain tissue

 Cerebral edema

 Mass effect: Distortion of intracranial contents

 Raised intracranial pressure: compress on vital structures, transtentorial herniation

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2
Q

Identify the most common sites of intracranial tumour development in adults and children.

A

Adults: above the tentorium in one or the other cerebral hemisphere (supratentorial)

Children: posterior fossa in midline: cerebellum / brainstem (infratentorial)

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3
Q

Classification of Intracranial tumours.

A

1) Primary tumours
a) Neuroectodermal/ neuroepithelial tumours
b) Tumours derived from other structures in the cranial cavity

2) Secondary/ metastatic tumours:
- Commonly from lung, breast, kidney and malignant melanoma

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4
Q

Location of occurrence and extent of involvement of astrocytomas?

A
  • Occur mainly in:
    i) Brainstem, cerebellum in posterior fossa (children)
    ii) Cerebral hemispheres (young adults) in frontal and temporal lobe

Diffuse involvement:

i) Cerebellar ones: often cystic, contain proteinaceous fluid
ii) diffusely infiltrates surrounding tissue

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5
Q

2 approaches to grading of astrocytoma?

A

1) scale 1-4 (WHO classification) with increasing anaplasia

2) Low-grade (i.e. well-differentiated) or High-grade (i.e. anaplastic, highly malignant) based on features

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6
Q

Prognosis and treatment of astrocytoma?

A

Tumour may undergo malignant (anaplastic) change often after 5-10 years

Treatment:

  • accessible to excision but often incomplete
  • chemotherapy / radiotherapy
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7
Q

Define the histological features that relate to different grades of astrocytoma?

A
  • Increased cellularity
  • Nuclear pleomorphism / hyperchromasia = grade 2
  • Mitosis = grade 3
  • Vascular endothelial proliferation, areas of necrosis = grade 4
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8
Q

Explain the difference in biologic malignancy and histologic malignancy of brain tumours?

A
  • Histologically ‘benign’/ well-differentiated neuroectodermal tumours
    e. g. astrocytomas, oligodendrogliomas and ependymomas

are rarely encapsulated and behave biologically malignant and diffusely infiltrate the surrounding tissue

or may advance to become histologically malignant too

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9
Q

Describe the pattern of mstastasis of histologically malignant neuroectodermal tumours?

A

Rarely metastasize outside CNS

spread within the brain and spinal by direct infiltration through brain tissue and by spread through the CSF

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10
Q

How do most histologically malignant neuroectodermal tumours exert their effects?

A

1) destruction of normal tissue
2) mass effect
3) obstruction of CSF flow which results in hydrocephalus

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11
Q

Normal function of oligodendrocytes?

A

form myelin sheath along fiber tract

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12
Q

Location of occurence, prognosis of oligodendroglioma?

A

Usually occur in the cerebral hemispheres of adults,: more commonly in the frontal and temporal lobes, uncommon in the occipital lobes

Favorable prognosis if well-differentiated (patients may survive for several years)

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13
Q

Describe microscopic and macroscopic appearance of oligodendroglioma?

A

Macroscopically:
 Purple-brown
 Relatively well-circumscribed (less infiltrative)
 Little necrosis

Microscopically:
 Calcification within the tumour
 Characteristic ‘box-like’ appearance of cells
 Clear halo (= cytoplasm) round the uniform round nucleus
 Well-marked cell border

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14
Q

Location of occurrence of Ependymomas?

A

Arise from ventricular lining (ependymal cells)

Many in brainstem (= vital site), cerebellum, 4th ventricle

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15
Q

Histological features of Ependymomas?

A

Histologically well-differentiated:

  • regular round nuclei
  • true rosettes with cuboidal tumour cells surrounding central lumen
  • Perivascular pseudo-rosettes = cells arranged around blood vessels = characteristic pink fibrillary halo around vessels
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16
Q

Prognosis and treatment of ependymomas?

A
  • slow growth rate and encapsulated&raquo_space; cure by surgical resection
  • Mostly located in brainstem & cerebellum&raquo_space; inaccessible to surgical excision&raquo_space; high morbidity, mortality
  • Does not respond well to chemotherapy / radiotherapy
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17
Q

Describe how Glioblastoma multiforme develops?

A

anaplastic gliomas with ambiguous origin

  • Primary: arise from undifferentiated glial cells
  • Secondary: Develop in pre-existing astrocytomas by progressive loss of differentiation
18
Q

Location of occurence, Histological features of Glioblastoma multiforme?

A
  • arise in cerebral hemispheres + infiltrate surrounding brain

Histological:

  • necrosis, haemorrhage, edema
  • Central necrosis surrounded by degenerate tumour and pseudo-palisades**
  • extensive nuclear, cytoplasmic pleomorphism, multinucleate cells, hyperchromasia
  • Active angiogenesis with conspicuous capillary endothelial proliferation&raquo_space; Glomeruloid body** (grade 4)
19
Q

Age of onset, prognosis and treatment of Glioblastoma multiforme?

A

Majority occur in patients > age 40

No cure

Survive <1 year

20
Q

How does Glioblastoma multiforme develop into ‘butterfly lesion’?

A

Infiltration follow white matter tracts (e.g. fornix, corpus callosum) to spread across hemisphere

> > infiltrate ventricular system and subarachnoid space

21
Q

Location of occurrence, age of onset and mode of spread of Medulloblastoma?

A

Arise in the vermis (in midline of cerebellum in posterior fossa)

Common tumours of childhood

seeds readily throughout CSF

22
Q

Histological features of medulloblastoma?

A
  • Small cells with densely staining ovoid nuclei, little cytoplasm
  • Mitoitic figures
  • Hyperchromatic nucleus
  • Homer Wright rosette formation:
     Nuclei arranged in a circle
     Fibrillary processes extend into centre
23
Q

Classification of medulloblastoma?

A

Subtypes by genetics:
 Sonic hedgehog-activated
+/-
 WNT-activated

24
Q

Age of onset, location of occurence of meningiomas?

A
  • older age groups, particularly in women

Mostly attached to:

  • Dura on the base of skull (parasagittal, falx cerebri, base of brain); or
  • Inner side of calvarium (surrounds the brain)
25
Q

Histological features of meningiomas?

A

 Well demarcated
 Nuclei = uniform in size; often 1-2 small nuclei, pale centres
 Cellular whorls **
 Small calcospherites / psammoma bodies** (calcium deposition)
 Fibrillary matrix

Maligant form: Characterized by necrosis, mitosis, infiltration of brain tissue

26
Q

Prognosis and treatment of meningiomas?

A

Well demarcated from underlying brain

surgical removal is not difficult = prognosis is always good

27
Q

Age of onset, Location of occurence of Schwannomas?

A

Usually occur in adults, more in females

benign tumours of Schwann cells in cranial nerves

> 60% cases: acoustic nerve (CN VIII)
(followed by trigeminal (CN V))

28
Q

Unique symptoms of Schwannomas compared to most intracranial tumours?

A

Patients may present with deafness, tinnitus

29
Q

Histological subtypes and features of Schwannomas?

A
  • smooth encapsulated surface
  • 2 main histological patterns usually seen intermingled: Antoni Type A and B

Antoni type A areas:

  • Sheets of spindle cells with elongated nuclei
  • Parallel nuclei in palisades

Antoni type B areas:

  • loosely packed regions
  • small nuclei
  • Foam cells
30
Q

What is bilateral acoustic schwannomas characterstic of?

A

characteristic of central type of Neurofibromatosis (genetic predisposition)

31
Q

Histological types of Astrocytoma? (4)

A

Histology: mostly mixture of:

  • Fibrillary (= most common: lots of processes)
  • Protoplasmic
  • Pilocytic (common in children in cerebellum)
  • Gemistocytic
32
Q

Which intracranial tumours have high incidence of IDH mutation?

A

Astrocytoma
Oligoastrocytoma
Oligodendroglioma

33
Q

Which intracranial tumours have high incidence of 1p/19q co-deletion?

A

Oligoastrocytoma

34
Q

List 5 supratentorial tumours in the cerebral hemisphere?

A

1) Cerebral astrocytomas (adults)
2) Glioblastomas
3) Oligodendrogliomas
4) Meningiomas
5) Metastatic tumours (= most common)

35
Q

List 2 intracranial tumours from the ventricular system?

A

 Ependymomas

 Choroid plexus papillomas

36
Q

List 2 intracranial tumours from the sella turcica?

A

 Pituitary adenomas

 Craniopharyngiomas

37
Q

Which intracranial tumours are most common in children?

A

Astrocytomas in the cerebellum and brain stem

Medulloblastoma

Ependymomas

38
Q

Name one intracranial tumour often found in the posteior fossa in children?

A

Pilocytic astrocytoma

39
Q

List 5 types of infratentorial intracranial tumours in adults?

A
  • Acoustic schwannomas
  • Brainstem gliomas
  • Haemangioblastoma
  • Meningioma
  • Metastatic tumours
40
Q

Cerebral astrocytomas and glioblastomas occur more in which brain region?

A

more commonly in the frontal and temporal lobes, and are uncommon in the occipital lobes

Same with oligodendrogliomas

41
Q

What type of metastatic intracranial tumour is common in Chinese females?

A

Metastatic choriocarcinoma